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Sample records for demyelinating disease masquerading

  1. Charcot-Marie-Tooth disease masquerading as acute demyelinating encephalomyelitis-like illness.

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    Kim, Gun-Ha; Kim, Kyoung Min; Suh, Sang-Il; Ki, Chang-Seok; Eun, Baik-Lin

    2014-07-01

    X-linked Charcot-Marie-Tooth disease (CMTX1) is a clinically heterogeneous hereditary motor and sensory neuropathy with X-linked transmission. Common clinical manifestations of CMTX1 disease, as in other forms of Charcot-Marie-Tooth (CMT) disease, are distal muscle wasting and weakness, hyporeflexia, distal sensory disturbance, and foot deformities. Mutations in the connexin-32 gene (gap junction protein β1 [GJB1]) are responsible for CMTX1 disease. In this report, we describe a patient with CMTX1 disease presenting with recurrent attacks of transient and episodic acute demyelinating encephalomyelitis (ADEM)-like symptoms without previous signs of lower extremity weakness or foot deformities; the patient, as well as his asymptomatic mother, exhibited a novel GJB1 mutation (p.Met1Ile). Differential diagnosis of recurrent and transient ADEM-like illness, if unexplained, should include the possibility of CMTX1 disease. Copyright © 2014 by the American Academy of Pediatrics.

  2. Inflammatory demyelinating pseudotumor with hemorrhage masquerading high grade cerebral neoplasm

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    Amit Agrawal

    2015-03-01

    Full Text Available Demyelinating pseudotumors are rare, benign, solitary intracranial space occupying lesions which masquerade cerebral neoplasms. Contrast MRI shows open ring enhancement which is fairly specific for this entity. Advanced MRI techniques like MR spectroscopy and magnetizing transfer techniques can help differentiating these lesions. NAA/Cr ratio is significantly elevated in central regions of demyelinating pseudotumors than in gliomas and other lesions. Presence of abundant foamy macrophages, lymphoid inflammatory infiltrates around blood vessels, sheets of gemistocytic astrocytes with well-developed processes, well defined border of the lesion absence of neovascularity and necrosis should help us diagnose demyelinating pseudotumor fairly confidently on histopathology.

  3. Unicentric Castleman’s Disease Masquerading Pancreatic Neoplasm

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    Saurabh Jain

    2012-01-01

    Full Text Available Castleman’s disease is a rare nonclonal proliferative disorder of the lymph nodes with an unknown etiology. Common locations of Castleman’s disease are mediastinum, neck, axilla, and abdomen. Castleman’s disease of a peripancreatic location masquerading as pancreatic neoplasm is an even rarer entity. On search of published data, we came across about 17 cases published on peripancreatic Castleman’s disease until now. Here we are reporting a case of retropancreatic Castleman's disease masquerading as retroperitoneal neoplasm in a 46-year-old male patient.

  4. Glutamate Receptors in Neuroinflammatory Demyelinating Disease

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    2006-01-01

    Full Text Available Multiple sclerosis (MS is a chronic demyelinating disease of the human central nervous system (CNS. The condition predominantly affects young adults and is characterised by immunological and inflammatory changes in the periphery and CNS that contribute to neurovascular disruption, haemopoietic cell invasion of target tissues, and demyelination of nerve fibres which culminate in neurological deficits that relapse and remit or are progressive. The main features of MS can be reproduced in the inducible animal counterpart, experimental autoimmune encephalomyelitis (EAE. The search for new MS treatments invariably employs EAE to determine drug activity and provide a rationale for exploring clinical efficacy. The preclinical development of compounds for MS has generally followed a conventional, immunotherapeutic route. However, over the past decade, a group of compounds that suppress EAE but have no apparent immunomodulatory activity have emerged. These drugs interact with the N-methyl-D-aspartate (NMDA and α -amino-3-hydroxy-5-isoxazolepropionic acid (AMPA/kainate family of glutamate receptors reported to control neurovascular permeability, inflammatory mediator synthesis, and resident glial cell functions including CNS myelination. The review considers the importance of the glutamate receptors in EAE and MS pathogenesis. The use of receptor antagonists to control EAE is also discussed together with the possibility of therapeutic application in demyelinating disease.

  5. Aortoiliac occlusive disease masquerading as cerebrovascular accident

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    Nandeesh B

    2007-01-01

    Full Text Available Acute aortoiliac occlusion is an unusual but potentially catastrophic condition causing acute limb ischemia and associated with early and high rates of mortality and morbidity. It is caused by either embolic occlusion of the infra renal aorta at the bifurcation or beyond or thrombosis of the abdominal aorta and its large terminal branches. Neurological symptoms are rare manifestation of acute aortoiliac occlusion and when neurological symptoms predominate, patients are mistakenly considered to have cerebrovascular event. We present a 60-year-old man with atherosclerotic thrombotic occlusion of the left common iliac artery causing acute painful monoplegia. We mistook the acute monoplegia due to acute limb ischemia for cerebrovascular accident. Pathologic examination revealed a firm thrombus occluding the origin of left common iliac artery and extending along the length of the vessel. Acute aortic iliac occlusion can masquerade as a cerebrovascular stroke and a thorough clinical evaluation and imaging studies allow early diagnosis and instituting life-saving treatment timely.

  6. Acute Demyelinating Disease after Oral Therapy with Herbal Extracts

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    Alex Kostianovsky

    2011-06-01

    Full Text Available Central nervous system demyelinating processes such as multiple sclerosis and acute disseminated encephalomyelitis constitute a group of diseases not completely understood in their physiopathology. Environmental and toxic insults are thought to play a role in priming autoimmunity. The aim of the present report is to describe a case of acute demyelinating disease with fatal outcome occurring 15 days after oral exposure to herbal extracts.

  7. Involvement of morbilliviruses in the pathogenesis of demyelinating disease

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    Sips, G. J.; Chesik, D.; Glazenburg, L.; Wilschut, J.; De Keyser, J.; Wilczak, N.

    2007-01-01

    Two members of the morbillivirus genus of the family Paramyxoviridae, canine distemper virus (CDV) and measles virus (MV), are well-known for their ability to cause a chronic demyelinating disease of the CNS in their natural hosts, dogs and humans, respectively. Both viruses have been studied for

  8. An Occult Malignancy Behind a Demyelinating Disease

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    Saberio Lo Presti MD

    2016-10-01

    Full Text Available We report a case of a 38-year-old man presenting with bilateral lower extremity weakness and paresthesias that progressed during a 4-month period to severe polyneuropathy forcing the patient to be bed bound. Throughout his multiple hospitalizations, he was treated erroneously for chronic inflammatory demyelinating polyneuropathy, without significant improvement in his symptoms. In addition, he developed hepatosplenomegaly (organomegaly; endocrinopathies such as diabetes mellitus, central hypogonadism, and hypothyroidism; monoclonal spike evidenced in the serum electrophoresis; and hyperpigmentation of skin, altogether consistent with POEMS syndrome. During his last hospitalization he developed excruciating pain on his left hip, and imaging revealed the presence of a 9 × 6 cm osteolytic mass with sclerotic rim in the left acetabulum. Biopsy of the mass confirmed an isolated IgG lambda plasmacytoma. The patient received radiation to his left acetabular lesion followed by left hip replacement. Subsequently, the patient underwent autologous bone marrow transplant. Eighteen months after his initial presentation, he had satisfactory clinical response and is functional without significant limitations. POEMS syndrome is a rare paraneoplastic syndrome secondary to an underlying plasma cell disorder, which can oftentimes be overlooked and misdiagnosed. The median age of presentation is 51 years, and only 31% of the cases occur in fairly young patients under the age of 45 as evidenced in this case. As clinicians, we should be aware of the constellation of features associated with POEMS syndrome and be able to recognize them promptly.

  9. OTORHINOLARYNGOLOGICAL DISEASES MASQUERADING AS ORBITAL TUMOURS

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    Sharmistha Behera

    2017-09-01

    Full Text Available BACKGROUND The close proximity of the orbit and the paranasal sinuses, both of which share more than two-thirds of common walls makes the orbit too susceptible to infections spreading from the paranasal sinuses. In any case of proptosis indicating an orbital tumour, extension from a PNS lesion should be ruled out. MATERIALS AND METHODS The study was a retrospective descriptive study. Data taken from the patient's medical record during the period of July 2014- June 2017. A total of 32 cases of proptosis were studied all of which were secondary to paranasal sinus pathology. These cases were subject to routine clinical examination and investigations including CT and MRI scan. Histopathological diagnosis was obtained by biopsy in appropriate cases. RESULTS Out of 32 patients, males were 68.75% (n=22 and females were 31.25% (n=10. The largest age group are in the age range 41-50 years (31.25%, n=10 and in age group 0-10 years (31.25%. Out of the whole, 13 (40.6% of them were due to sinusitis, 6 (18.75% due to sinonasal malignancy, all of which were histopathologically confirmed to be squamous cell carcinoma of maxillary sinus, 6 (18.75% cases were of mucoceles from frontoethmoidal origin, 4 (12.50% cases were of fibrous dysplasia of maxillary sinus, 2 (6.25% case was of schwannoma of frontal sinus origin and 1 (3.12% case of Langerhans cell histiocytosis of maxillary sinus. In our study, proptosis was commonest presenting complaint in all 32 patients followed by nasal obstruction (62.5%, reduced vision (25%, facial asymmetry (25%, redness of eye (18.75% and double vision (12.50%. Out of all investigative modalities, biopsy was found to be the most accurate followed by CT scan brain, PNS and orbit. CONCLUSION Due to close proximity, nasal and PNS diseases through bone erosion or preformed pathways can invade the orbit and cause proptosis. Longstanding optic nerve compression by orbital encroachment of PNS lesion can lead to blindness due to optic

  10. Demyelination load as predictor for disease progression in juvenile metachromatic leukodystrophy.

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    Strölin, Manuel; Krägeloh-Mann, Ingeborg; Kehrer, Christiane; Wilke, Marko; Groeschel, Samuel

    2017-06-01

    The aim of this study was to investigate whether the extent and topography of cerebral demyelination correlates with and predicts disease progression in patients with juvenile metachromatic leukodystrophy (MLD). A total of 137 MRIs of 46 patients with juvenile MLD were analyzed. Demyelination load and brain volume were quantified using the previously developed Software "clusterize." Clinical data were collected within the German Leukodystrophy Network and included full scale intelligence quotient (FSIQ) and gross motor function data. Voxel-based lesion-symptom mapping (VLSM) across the whole brain was performed to investigate the spatial relationship of cerebral demyelination with motor or cognitive function. The prognostic value of the demyelination load at disease onset was assessed to determine the severity of disease progression. The demyelination load (corrected by the individual brain volume) correlated significantly with gross motor function ( r = +0.55) and FSIQ ( r = -0.55). Demyelination load at disease onset was associated with the severity of disease progression later on ( P loss in FSIQ and more central region demyelination with decline of motor function. Especially progression of demyelination within the motor area was associated with severe disease progression. We were able to show for the first time in a large cohort of patients with juvenile MLD that the demyelination load correlates with motor and cognitive symptoms. Moreover, demyelination load at disease onset, especially the involvement of the central region, predicts severity of disease progression. Thus, demyelination load seems a functionally relevant MRI parameter.

  11. Myelin Oligodendrocyte Glycoprotein: Deciphering a Target in Inflammatory Demyelinating Diseases

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    Patrick Peschl

    2017-05-01

    Full Text Available Myelin oligodendrocyte glycoprotein (MOG, a member of the immunoglobulin (Ig superfamily, is a myelin protein solely expressed at the outermost surface of myelin sheaths and oligodendrocyte membranes. This makes MOG a potential target of cellular and humoral immune responses in inflammatory demyelinating diseases. Due to its late postnatal developmental expression, MOG is an important marker for oligodendrocyte maturation. Discovered about 30 years ago, it is one of the best-studied autoantigens for experimental autoimmune models for multiple sclerosis (MS. Human studies, however, have yielded controversial results on the role of MOG, especially MOG antibodies (Abs, as a biomarker in MS. But with improved detection methods using different expression systems to detect Abs in patients’ samples, this is meanwhile no longer the case. Using cell-based assays with recombinant full-length, conformationally intact MOG, several recent studies have revealed that MOG Abs can be found in a subset of predominantly pediatric patients with acute disseminated encephalomyelitis (ADEM, aquaporin-4 (AQP4 seronegative neuromyelitis optica spectrum disorders (NMOSD, monophasic or recurrent isolated optic neuritis (ON, or transverse myelitis, in atypical MS and in N-methyl-d-aspartate receptor-encephalitis with overlapping demyelinating syndromes. Whereas MOG Abs are only transiently observed in monophasic diseases such as ADEM and their decline is associated with a favorable outcome, they are persistent in multiphasic ADEM, NMOSD, recurrent ON, or myelitis. Due to distinct clinical features within these diseases it is controversially disputed to classify MOG Ab-positive cases as a new disease entity. Neuropathologically, the presence of MOG Abs is characterized by MS-typical demyelination and oligodendrocyte pathology associated with Abs and complement. However, it remains unclear whether MOG Abs are a mere inflammatory bystander effect or truly pathogenetic

  12. Cerebrospinal fluid analysis in the context of CNS demyelinating diseases

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    Sandro Luiz de Andrade Matas

    2013-09-01

    Full Text Available The central nervous system demyelinating diseases are a group of disorders with different etiologies, characterized by inflammatory lesions that are associated with loss of myelin and eventually axonal damage. In this group the most studied ones are multiple sclerosis (MS, neuromyelitis optic (NMO and acute disseminated encephalomyelitis (ADEM. The cerebrospinal fluid is essential to differentiate between these different syndromes and to define multiple sclerosis, helping to assess the probability of Clinical Isolated Syndrome turn into multiple sclerosis.

  13. [Recent progress in multiple sclerosis research: astrocytopathy in demyelinating diseases].

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    Kira, Jun-Ichi

    2010-11-01

    Multiple sclerosis (MS) is a demyelinating disease of the central nervous system (CNS) while neuromyelitis optica (NMO) is an inflammatory disease of the CNS that selectively affects the optic nerves and spinal cord. Recently, a specific IgG against NMO, designated NMO-IgG, was discovered, and the relevant antigen was found to be aquaporin 4 (AQP4), one of the major water channel proteins in the CNS. The sensitivity of NMO-IgG/anti-AQP4 antibodies for NMO varies from 30% to 80%, while specificity is 90-100%. Pathological studies on NMO patients have revealed perivascular immune complex (IgM, IgG and C9neo) deposition and extensive loss of AQP4 in active lesions, while myelin basic protein (MBP) staining was relatively preserved. IgG from NMO-IgG-seropositive NMO patients induces astrocyte death in culture in the presence of complement, and reproduces astrocyte loss in vivo when MBP-specific T cells are co-transferred to cause experimental autoimmune encephalomyelitis. Therefore, it is postulated that the complement-activating anti-AQP4 antibodies have a pivotal role in the development of NMO lesions through astrocyte necrosis, and that demyelination is a secondary event. Baló's disease is characterized by alternating rings of demyelination and preserved myelin. As additional MS-like lesions often coexist in Baló's cases, Baló's disease is regarded as a variant of MS. However, Baló's concentric rings are also observed in NMO cases and in Asian opticospinal MS patients in the cerebral white matter, spinal cord and optic chiasm. In demyelinated areas, many hypertrophic astrocytes are present, in close contact with oligodendrocytes that often show apoptotic features. In the outermost layer of preserved myelin, stress proteins involved in tissue preconditioning are abundant in oligodendrocytes. The peri-plaque white matter is thus assumed resistant to subsequent attack, thereby leaving a layer of preserved myelin. In some patients, Baló's concentric rings develop

  14. Citation classics in central nervous system inflammatory demyelinating disease.

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    Kim, Jee-Eun; Park, Kang M; Kim, Yerim; Yoon, Dae Y; Bae, Jong S

    2017-06-01

    To identify and analyze the characteristics of the most influential articles about central nervous system (CNS) inflammatory demyelinating disease. The Institute for Scientific Information (ISI) Web of Science database and the 2014 Journal Citation Reports Science Edition were used to retrieve the top 100 cited articles on CNS inflammatory demyelinating disease. The citation numbers, journals, years of publication, authorships, article types, subjects and main issues were analyzed. For neuromyelitis optica (NMO), articles that were cited more than 100 times were regarded as a citation classic and described separately. The top 100 cited articles were published between 1972 and 2011 in 13 journals. The highest number of articles ( n  = 24) was published in Brain, followed by The New England Journal of Medicine ( n  = 21). The average number of citations was 664 (range 330-3,897), and 64% of the articles were from the United States and the United Kingdom. The majority of the top 100 cited articles were related to multiple sclerosis ( n  = 87), and only a few articles reported on other topics such as NMO ( n  = 9), acute disseminated encephalomyelitis ( n  = 2) and optic neuritis ( n  = 2). Among the top 100 cited articles, 77% were original articles. Forty-one citation classics were found for NMO. Our study provides a historical perspective on the research progress on CNS inflammatory demyelinating disease and may serve as a guide for important advances and trends in the field for associated researchers.

  15. Imaging of demyelinating and degenerative diseases of the brain

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    Drayer, B.P.

    1987-01-01

    The emergence of cross-sectional brain imaging in the past decade has greatly expanded the role of imaging as a primary diagnostic modality for demyelinating and degenerative brain disorders. To remain an effective neurologic consultant, the radiologist must better understand the neuropathology and functional significance of these disorders. MR imaging has become the dominant imaging modality for multiple sclerosis and all demyelinating and dysmyelinating disorders. Detection is most sensitive with intermediate and T2-weighted spin-echo pulse sequences. Although increased signal intensity in the white matter is a sensitive but nonspecific finding, a knowledge of the patient's history and disease pathoanatomy greatly improves diagnostic specificity. Since an increasing proportion of the population is over 65 years of age, the distinction of normal versus pathologic aging becomes critical. The role of imaging in dementing illness is to distinguish primary degenerative dementia from normal aging changes, vascular medullary artery distribution disease, microangiopathic leukoencephalopathy, communicating hydrocephalus, and mass lesions. The role of MR imaging, including brain iron mapping, is analyzed in bradykinetic, choreiform, and dystonic disorders. The complications of chronic ethanol abuse, including vermian atrophy, central pontine myelinolysis, and Wernicke encephalopathy, are also reviewed

  16. A 17 year-old girl with a demyelinating disease requiring mechanical ventilation: a case report

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    Katsenos Chrysostomos

    2013-01-01

    Full Text Available Abstract Background Demyelinating diseases cause destruction of the myelin sheath, while axons are relatively spared. Pathologically, demyelination can be the result of an inflammatory process, viral infection, acquired metabolic derangement and ischemic insult. Three diseases that can cause inflammatory demyelination of the CNS are: Multiple sclerosis (MS, Acute disseminated encephalomyelitis (ADEM and Acute hemorrhagic leucoencephalitis. Differentiation is not always easy and there is considerable overlaping. Data about adults with acute demyelination requiring ICU admission is limited. Case presentation A 17 year old Greek female was hospitalised in the ICU because of acute respiratory failure requiring mechanical ventilation. She had a history of febrile disease one month before, acute onset of paraplegia, diplopia, progressive arm weakness and dyspnea. Her consciousness was not impaired. A demyelinating central nervous system (CNS disease, possibly post infectious encephalomyelitis (ADEM was the underlying condition. The MRI of the brain disclosed diffused expanded cerebral lesions involving the optic nerve, basal ganglia cerebellum, pons and medulla oblongata. There was also extended involvement of the cervical and thoracic part of the spinal cord. CSF leukocyte count was elevated with lymphocyte predominance. The patient required mechanical ventilation for two months. Then she was transferred to a rehabilitation centre. Three years later she remains paraplegic. Since then she has not suffered any other demyelination attack. Conclusions Demyelinating diseases can cause acute respiratory failure when the spinal cord is affected. Severe forms of these diseases, making necessary ICU admission, is less frequently reported. Intensivists should be aware of the features of these rare diseases.

  17. Dysregulation of ErbB Receptor Trafficking and Signaling in Demyelinating Charcot-Marie-Tooth Disease.

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    Lee, Samuel M; Chin, Lih-Shen; Li, Lian

    2017-01-01

    Charcot-Marie-Tooth (CMT) disease is the most common inherited peripheral neuropathy with the majority of cases involving demyelination of peripheral nerves. The pathogenic mechanisms of demyelinating CMT remain unclear, and no effective therapy currently exists for this disease. The discovery that mutations in different genes can cause a similar phenotype of demyelinating peripheral neuropathy raises the possibility that there may be convergent mechanisms leading to demyelinating CMT pathogenesis. Increasing evidence indicates that ErbB receptor-mediated signaling plays a major role in the control of Schwann cell-axon communication and myelination in the peripheral nervous system. Recent studies reveal that several demyelinating CMT-linked proteins are novel regulators of endocytic trafficking and/or phosphoinositide metabolism that may affect ErbB receptor signaling. Emerging data have begun to suggest that dysregulation of ErbB receptor trafficking and signaling in Schwann cells may represent a common pathogenic mechanism in multiple subtypes of demyelinating CMT. In this review, we focus on the roles of ErbB receptor trafficking and signaling in regulation of peripheral nerve myelination and discuss the emerging evidence supporting the potential involvement of altered ErbB receptor trafficking and signaling in demyelinating CMT pathogenesis and the possibility of modulating these trafficking and signaling processes for treating demyelinating peripheral neuropathy.

  18. Idiopathic inflammatory-demyelinating diseases of the central nervous system

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    Rovira Canellas, A. [Vall d' Hebron University Hospital, Magnetic Resonance Unit (I.D.I.), Department of Radiology, Barcelona (Spain); Rovira Gols, A. [Parc Tauli University Institute - UAB, UDIAT, Diagnostic Centre, Sabadell (Spain); Rio Izquierdo, J.; Tintore Subirana, M.; Montalban Gairin, X. [Vall d' Hebron University Hospital, Neuroimmunology Unit, Department of Neurology, Barcelona (Spain)

    2007-05-15

    Idiopathic inflammatory-demyelinating diseases (IIDDs) include a broad spectrum of central nervous system disorders that can usually be differentiated on the basis of clinical, imaging, laboratory and pathological findings. However, there can be a considerable overlap between at least some of these disorders, leading to misdiagnoses or diagnostic uncertainty. The relapsing-remitting and secondary progressive forms of multiple sclerosis (MS) are the most common IIDDs. Other MS phenotypes include those with a progressive course from onset (primary progressive and progressive relapsing) or with a benign course continuing for years after onset (benign MS). Uncommon forms of IIDDs can be classified clinically into: (1) fulminant or acute IIDDs, such as the Marburg variant of MS, Balo's concentric sclerosis, Schilder's disease, and acute disseminated encephalomyelitis; (2) monosymptomatic IIDDs, such as those involving the spinal cord (transverse myelitis), optic nerve (optic neuritis) or brainstem and cerebellum; and (3) IIDDs with a restricted topographical distribution, including Devic's neuromyelitis optica, recurrent optic neuritis and relapsing transverse myelitis. Other forms of IIDD, which are classified clinically and radiologically as pseudotumoral, can have different forms of presentation and clinical courses. Although some of these uncommon IIDDs are variants of MS, others probably correspond to different entities. MR imaging of the brain and spine is the imaging technique of choice for diagnosing these disorders, and together with the clinical and laboratory findings can accurately classify them. Precise classification of these disorders may have relevant prognostic and treatment implications, and might be helpful in distinguishing them from tumoral or infectious lesions, avoiding unnecessary aggressive diagnostic or therapeutic procedures. (orig.)

  19. Idiopathic inflammatory-demyelinating diseases of the central nervous system

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    Rovira Canellas, A.; Rovira Gols, A.; Rio Izquierdo, J.; Tintore Subirana, M.; Montalban Gairin, X.

    2007-01-01

    Idiopathic inflammatory-demyelinating diseases (IIDDs) include a broad spectrum of central nervous system disorders that can usually be differentiated on the basis of clinical, imaging, laboratory and pathological findings. However, there can be a considerable overlap between at least some of these disorders, leading to misdiagnoses or diagnostic uncertainty. The relapsing-remitting and secondary progressive forms of multiple sclerosis (MS) are the most common IIDDs. Other MS phenotypes include those with a progressive course from onset (primary progressive and progressive relapsing) or with a benign course continuing for years after onset (benign MS). Uncommon forms of IIDDs can be classified clinically into: (1) fulminant or acute IIDDs, such as the Marburg variant of MS, Balo's concentric sclerosis, Schilder's disease, and acute disseminated encephalomyelitis; (2) monosymptomatic IIDDs, such as those involving the spinal cord (transverse myelitis), optic nerve (optic neuritis) or brainstem and cerebellum; and (3) IIDDs with a restricted topographical distribution, including Devic's neuromyelitis optica, recurrent optic neuritis and relapsing transverse myelitis. Other forms of IIDD, which are classified clinically and radiologically as pseudotumoral, can have different forms of presentation and clinical courses. Although some of these uncommon IIDDs are variants of MS, others probably correspond to different entities. MR imaging of the brain and spine is the imaging technique of choice for diagnosing these disorders, and together with the clinical and laboratory findings can accurately classify them. Precise classification of these disorders may have relevant prognostic and treatment implications, and might be helpful in distinguishing them from tumoral or infectious lesions, avoiding unnecessary aggressive diagnostic or therapeutic procedures. (orig.)

  20. Association between demyelinating disease and autoimmune rheumatic disease in a pediatric population.

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    Amorim, Ana Luiza M; Cabral, Nadia C; Osaku, Fabiane M; Len, Claudio A; Oliveira, Enedina M L; Terreri, Maria Teresa

    Multiple sclerosis (MS) and neuromyelitis optica (NMO) are demyelinating diseases of the central nervous system. Autoimmunity in patients with demyelinating disease and in their families has been broadly investigated and discussed. Recent studies show a higher incidence of rheumatic autoimmune diseases among adult patients with MS or NMO and their families, but there are no studies in the pediatric population. To evaluate an association of MS and NMO with autoimmune rheumatic diseases in pediatric patients. 22 patients younger than 21 years old with MS or NMO diagnosed before the age of 18 years were evaluated regarding epidemiological data, clinical presentation, association with autoimmune diseases, family history of autoimmune diseases, laboratory findings, imaging studies and presence of auto-antibodies. Among the patients studied, there was a prevalence of females (68.1%). The mean age of symptoms onset was 8 years and 9 months and the mean current age was 16 years and 4 months. Two patients (9%) had a history of associated autoimmune rheumatic disease: one case of juvenile dermatomyositis in a patient with NMO and another of systemic lupus erythematosus in a patient with MS. Three patients (13%) had a family history of autoimmunity in first-degree relatives. Antinuclear antibody was found positive in 80% of patients with NMO and 52% of patients with MS. About 15% of antinuclear antibody-positive patients were diagnosed with rheumatologic autoimmune diseases. Among patients with demyelinating diseases diagnosed in childhood included in this study there was a high frequency of antinuclear antibody positivity but a lower association with rheumatologic autoimmune diseases than that observed in studies conducted in adults. Copyright © 2016 Elsevier Editora Ltda. All rights reserved.

  1. Pain and spinal cord imaging measures in children with demyelinating disease

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    Nadia Barakat

    2015-01-01

    Full Text Available Pain is a significant problem in diseases affecting the spinal cord, including demyelinating disease. To date, studies have examined the reliability of clinical measures for assessing and classifying the severity of spinal cord injury (SCI and also to evaluate SCI-related pain. Most of this research has focused on adult populations and patients with traumatic injuries. Little research exists regarding pediatric spinal cord demyelinating disease. One reason for this is the lack of reliable and useful approaches to measuring spinal cord changes since currently used diagnostic imaging has limited specificity for quantitative measures of demyelination. No single imaging technique demonstrates sufficiently high sensitivity or specificity to myelin, and strong correlation with clinical measures. However, recent advances in diffusion tensor imaging (DTI and magnetization transfer imaging (MTI measures are considered promising in providing increasingly useful and specific information on spinal cord damage. Findings from these quantitative imaging modalities correlate with the extent of demyelination and remyelination. These techniques may be of potential use for defining the evolution of the disease state, how it may affect specific spinal cord pathways, and contribute to the management of pediatric demyelination syndromes. Since pain is a major presenting symptom in patients with transverse myelitis, the disease is an ideal model to evaluate imaging methods to define these regional changes within the spinal cord. In this review we summarize (1 pediatric demyelinating conditions affecting the spinal cord; (2 their distinguishing features; and (3 current diagnostic and classification methods with particular focus on pain pathways. We also focus on concepts that are essential in developing strategies for the detection, monitoring, treatment and repair of pediatric myelitis.

  2. Plasminogen Deficiency Delays the Onset and Protects from Demyelination and Paralysis in Autoimmune Neuroinflammatory Disease.

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    Shaw, Maureen A; Gao, Zhen; McElhinney, Kathryn E; Thornton, Sherry; Flick, Matthew J; Lane, Adam; Degen, Jay L; Ryu, Jae Kyu; Akassoglou, Katerina; Mullins, Eric S

    2017-04-05

    Multiple sclerosis (MS) is a neuroinflammatory, demyelinating disease of the CNS. Fibrinogen deposition at sites of blood-brain barrier breakdown is a prominent feature of neuroinflammatory disease and contributes to disease severity. Plasminogen, the primary fibrinolytic enzyme, also modifies inflammatory processes. We used a murine model of MS, experimental autoimmune encephalomyelitis (EAE), to evaluate the hypothesis that the loss of plasminogen would exacerbate neuroinflammatory disease. However, contrary to initial expectations, EAE-challenged plasminogen-deficient (Plg - ) mice developed significantly delayed disease onset and reduced disease severity compared with wild-type (Plg + ) mice. Similarly, pharmacologic inhibition of plasmin activation with tranexamic acid also delayed disease onset. The T-cell response to immunization was similar between genotypes, suggesting that the contribution of plasminogen was downstream of the T-cell response. Spinal cords from EAE-challenged Plg - mice demonstrated significantly decreased demyelination and microglial/macrophage accumulation compared with Plg + mice. Although fibrinogen-deficient mice or mice with combined deficiencies of plasminogen and fibrinogen had decreased EAE severity, they did not exhibit the delay in EAE disease onset, as seen in mice with plasminogen deficiency alone. Together, these data suggest that plasminogen and plasmin-mediated fibrinolysis is a key modifier of the onset of neuroinflammatory demyelination. SIGNIFICANCE STATEMENT Multiple sclerosis is a severe, chronic, demyelinating disease. Understanding the pathobiology related to the autoreactive T-cell and microglial/macrophage demyelinating response is critical to effectively target therapeutics. We describe for the first time that deficiency of plasminogen, the key fibrinolytic enzyme, delays disease onset and protects from the development of the paralysis associated with a murine model of multiple sclerosis, experimental autoimmune

  3. Psychiatric disorders in children with demyelinating diseases of the central nervous system.

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    Pakpoor, Julia; Goldacre, Raph; Schmierer, Klaus; Giovannoni, Gavin; Waubant, Emmanuelle; Goldacre, Michael J

    2017-07-01

    The profile of psychiatric disorders associated with multiple sclerosis (MS) may differ in children. We aimed to assess the risk of psychiatric disorders in children with MS and other demyelinating diseases, and vice versa. We analyzed linked English Hospital Episode Statistics, and mortality data, 1999-2011. Cohorts were constructed of children admitted with MS and other central nervous system (CNS) demyelinating diseases. We searched for any subsequent episode of care with psychiatric disorders in these cohorts and compared to a reference cohort. Children with CNS demyelinating diseases had an increased rate of psychotic disorders (rate ratio (RR) = 5.77 (95% confidence interval (CI) = 2.48-11.41)); anxiety, stress-related, and somatoform disorders (RR = 2.38 (1.39-3.81)); intellectual disability (RR = 6.56 (3.66-10.84)); and other behavioral disorders (RR = 8.99 (5.13-14.62)). In analysis of the pediatric MS cohort as the exposure, there were elevated rates of psychotic disorders (RR = 10.76 (2.93-27.63)), mood disorders (RR = 2.57 (1.03-5.31)), and intellectual disability (RR = 6.08 (1.25-17.80)). In reverse analyses, there were elevated rates of a recorded hospital episode with CNS demyelinating disease after a previous recorded episode with anxiety, stress-related, and somatoform disorders; attention-deficit hyperactivity disorder (ADHD); autism; intellectual disability; and other behavioral disorders. This analysis of a national diagnostic database provides strong evidence for an association between pediatric CNS demyelinating diseases and psychiatric disorders, and highlights a need for early involvement of mental health professionals.

  4. How not to miss autoinflammatory diseases masquerading as urticaria

    DEFF Research Database (Denmark)

    Krause, K; Grattan, C E; Bindslev-Jensen, C

    2012-01-01

    symptoms including recurrent fever attacks, arthralgia or arthritis and fatigue. Autoinflammatory diseases are often associated with a diagnostic delay of many years and do not respond to antihistamines and other treatments of urticaria. Also, the chronic inflammation may lead to long-term complications...

  5. A Pathogenic Role for CD8+ T Cells in a Spontaneous Model of Demyelinating Disease

    DEFF Research Database (Denmark)

    Brisebois, Marcel; Zehntner, Simone P.; Estrada, José

    2006-01-01

    Transgenic (Tg) mice that overexpress the costimulatory ligand B7.2/CD86 on microglia spontaneously develop a T cell-mediated demyelinating disease. Characterization of the inflammatory infiltrates in the nervous tissue revealed a predominance of CD8+ T cells, suggesting a prominent role of this T...... cell subset in the pathology. In this study, we show that the same neurological disease occurred in Tg mice deficient in the generation of CD4+ T cells, with an earlier time of onset. Analysis of the CD8+ T cell repertoire at early stage of disease revealed the presence of selected clonal expansions...... pathogenesis. Collectively, our data indicate that the spontaneous demyelinating disease in this animal model occurs as a consequence of an inflammatory response initiated through the activation of CNS-specific CD8+ T cells by Tg expression of B7.2 within the target organ. Thus, autoreactive CD8+ T cells can...

  6. Progressive wheeze: atrial myxoma masquerading as chronic obstructive pulmonary disease.

    Science.gov (United States)

    Sinha, Aish; Apps, Andrew; Liong, Wei Chuen; Firoozan, Soroosh

    2015-07-23

    Atrial myxoma, the commonest primary cardiac neoplasm, presents with symptoms of heart failure, embolic phenomena or constitutional upset. We present an atypical case, with wheeze and symptomatic exacerbations typical of chronic obstructive pulmonary disease. With no early clinical evidence of heart failure, the patient was managed with inhaled steroids and bronchodilators, with little relief. Only when the patient was in extremis requiring intubation, due to respiratory failure, did clinical evidence of left heart failure become apparent, with echocardiography demonstrating a massive left atrial myxoma obstructing the mitral valve annulus. Following successful surgical resection, the patient's symptoms fully abated. This case highlights the importance of considering cardiac wheeze in those initially managed as obstructive airway disease not responding in a typical fashion to initial bronchodilator therapy, and particularly in those with rapidly progressive symptoms. Such patients should be referred early for cardiac imaging. The excellent prognosis and quick recovery after timely surgical resection of a myxoma are also highlighted. 2015 BMJ Publishing Group Ltd.

  7. Viridans streptococci Intracranial Abscess Masquerading as Metastatic Disease

    Directory of Open Access Journals (Sweden)

    Brandon Ruderman

    2018-01-01

    Full Text Available History of present illness: A 64-year-old woman with a remote history of breast cancer presented to the emergency department with one day of worsening right-sided weakness and headaches. She had been seen two weeks prior for similar symptoms and underwent unenhanced brain computed tomography (CT, which revealed a left parietal mass suspected to represent new metastatic disease. The patient presented on this visit not only with weakness of her right upper and lower extremity, but was found to be febrile, tachycardic and exhibiting lethargy. Peripheral white blood cell count (WBC was 27.1 (x109/L. Given her rapidly declining clinical course and expected time to complete magnetic resonance imaging (MRI of the brain, the decision was made to first perform an emergent contrast-enhanced brain CT to further evaluate the mass. The contrast-enhanced brain CT revealed a large rim-enhancing left parietal lesion (Figures 1 and 2. Significant findings: A non-contrast CT (Figure 1 revealed a large hypoattenuating left parietal lesion. When the CT was enhanced with intravenous contrast (Figure 2, the same lesion showed peripheral rim enhancement, suggestive of a brain abscess. Discussion: Intracranial abscess is a rare yet potentially devastating disease process that can often be difficult to diagnosis, with an incidence of about 0.4-0.9 cases per 100,000 people.1 Mortality rates are about 15% within the past decade, but can increase up to 85% if the abscess ruptures. The most common mechanisms for formation of intracranial abscesses include direct spread from local infections (odontogenic infections or sinusitis, hematogenous spread (congenital heart disease, endocarditis, dental procedures, or intrapulmonary shunting, or penetrating trauma.2 Intracranial abscesses are more likely to occur in severely immunosuppressed patients, after neurosurgical procedures, or in head trauma. About 32%-60% of brain abscesses are polymicrobial, but Viridans streptococci, a

  8. Demyelinating and ischemic brain diseases: detection algorithm through regular magnetic resonance images

    Science.gov (United States)

    Castillo, D.; Samaniego, René; Jiménez, Y.; Cuenca, L.; Vivanco, O.; Rodríguez-Álvarez, M. J.

    2017-09-01

    This work presents the advance to development of an algorithm for automatic detection of demyelinating lesions and cerebral ischemia through magnetic resonance images, which have contributed in paramount importance in the diagnosis of brain diseases. The sequences of images to be used are T1, T2, and FLAIR. Brain demyelination lesions occur due to damage of the myelin layer of nerve fibers; and therefore this deterioration is the cause of serious pathologies such as multiple sclerosis (MS), leukodystrophy, disseminated acute encephalomyelitis. Cerebral or cerebrovascular ischemia is the interruption of the blood supply to the brain, thus interrupting; the flow of oxygen and nutrients needed to maintain the functioning of brain cells. The algorithm allows the differentiation between these lesions.

  9. Demyelinating syndrome in SLE: review of different disease subtypes and report of a case series

    Directory of Open Access Journals (Sweden)

    E. Chessa

    2017-12-01

    Full Text Available Demyelinating syndrome (DS is a rare manifestation of systemic lupus erythematosus (SLE (1% with high clinical heterogeneity and potentially severe prognosis. It can represent a diagnostic and therapeutic challenge for clinicians. A recent study described 5 different patterns of demyelinating disease presentation, characterised by specific clinical, laboratory and brain and spine magnetic resonance imaging abnormalities: 1 neuromyelitis optica; 2 neuromyelitis optica spectrum disorders; 3 DS prevalently involving the brain; 4 DS prevalently involving the brainstem; 5 clinically isolated syndrome. In this review we briefly discuss typical characteristics of each DS presentation in SLE and we describe 5 illustrative clinical cases, one for each subset of DS, considering both diagnostic and therapeutic options.

  10. [Pseudotumor forms of demyelinating diseases. Report of three cases and review of the literature].

    Science.gov (United States)

    de Lacour, A; Guisado, F; Zambrano, A; Argente, J; Acosta, J; Ramos, C

    1998-12-01

    We present three patients with unusual clinical findings studied in our hospital, after a period of follow-up of at least two years from the time of diagnosis of their demyelinating condition. We discuss the clinical onset, CT and magnetic resonance findings, neuropathological studies and posterior clinical course. Anatomopathological studies were done in two of the cases, since the diagnoses were not clear and the other paraclinical investigations did not clarify things. The presence of large space-occupying lesions or ring-like outlines in myelinating disorders may make it difficult to make a differential diagnosis from other conditions such as neoplasias and abscesses. This may lead to an erroneous initial diagnosis and even to the use of unsuitable, aggressive treatments. In young patients in whom radiological imaging suggests space-occupying lesions (single or multiple) one should consider the possibility of a primary demyelinating disease of the central nervous system with the appearance of a pseudo-tumour. In certain cases, stereotaxic biopsy should be considered if the diagnosis remains in doubt, rather than begin unsuitable treatment. The fundamental reason for the presentation of our cases is to emphasize that these pseudo-tumoral forms of demyelinating diseases should be considered in diagnosis.

  11. CD8+ T cells in inflammatory demyelinating disease

    DEFF Research Database (Denmark)

    Weiss, Hanne A; Millward, Jason M; Owens, Trevor

    2007-01-01

    We review the contribution made by CD8+ T cells to inflammation in the central nervous system (CNS) in Multiple Sclerosis (MS), and discuss their role in the animal model Experimental Autoimmune Encephalomyelitis (EAE). We show that the inflammatory cytokines interferon-gamma and interleukin-17...... are differentially regulated in CNS-infiltrating CD4+ and CD8+ T cells in EAE, and that CD8+ T cells regulate disease. In MS, CD8+ T cells appear to play a role in promotion of disease, so cytokine regulation is likely different in CD8+ T cells in MS and EAE...

  12. Demyelination in mild cognitive impairment suggests progression path to Alzheimer's disease.

    Directory of Open Access Journals (Sweden)

    Cristian Carmeli

    Full Text Available The preclinical Alzheimer's disease (AD - amnestic mild cognitive impairment (MCI - is manifested by phenotypes classified into exclusively memory (single-domain MCI (sMCI and multiple-domain MCI (mMCI. We suggest that typical MCI-to-AD progression occurs through the sMCI-to-mMCI sequence as a result of the extension of initial pathological processes. To support this hypothesis, we assess myelin content with a Magnetization Transfer Ratio (MTR in 21 sMCI and 21 mMCI patients and in 42 age-, sex-, and education-matched controls. A conjunction analysis revealed MTR reduction shared by sMCI and mMCI groups in the medial temporal lobe and posterior structures including white matter (WM: splenium, posterior corona radiata and gray matter (GM: hippocampus; parahippocampal and lingual gyri. A disjunction analysis showed the spread of demyelination to prefrontal WM and insula GM in executive mMCI. Our findings suggest that demyelination starts in the structures affected by neurofibrillary pathology; its presence correlates with the clinical picture and indicates the method of MCI-to-AD progression. In vivo staging of preclinical AD can be developed in terms of WM/GM demyelination.

  13. Diffusion abnormality maps in demyelinating disease: correlations with clinical scores.

    Science.gov (United States)

    Onu, Mihaela; Roceanu, Adina; Sboto-Frankenstein, Uta; Bendic, Robert; Tarta, Eugen; Preoteasa, Florentin; Bajenaru, Ovidiu

    2012-03-01

    Magnetic resonance imaging (MRI) has been explored as a noninvasive tool to assess pathology in multiple sclerosis (MS) patients. However, the correlation between classical MRI measures and physical disability is modest in MS. The diffusion tensor imaging (DTI) MRI technique holds particular promise in this regard. The present study shows brain regions where FA and individual diffusivities abnormalities are present and check their correlations with physical disability clinical scores. Eight patients and 12 matched healthy controls were recruited. The Multiple Sclerosis Functional Composite was administered. For MR-DTI acquisitions, a Genesis Signa 1.5 T MR system, an EP/SE scanning sequence, 25 gradient directions were used. Tract Based Spatial Statistics (TBSS) group comparisons showed reduced FA and increased individual diffusivities in several brain regions in patients. Significant correlations were found between FA and: EDSS, 9-HPT(NON)DOM and 25 FW score; between λ2 and: P100 (r&l), 9-HPT(NON)DOM and 25 FW; between λ3 and: 9-HPT(NON)DOM and 25 FW score. Fractional anisotropy and individual radial diffusivities proved to be important markers of motor disabilities in MS patients when the disease duration mean and the disability scores values range are relatively high. Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.

  14. Gray Matter Changes in Demyelinating Disease: Correlations with Clinical Scores.

    Science.gov (United States)

    Onu, Mihaela; Aroceanu, Adina; Ferastraoaru, Victor; Bajenaru, Ovidiu

    2015-09-01

    Recent MR studies have shown that, in multiple sclerosis, selective regional, but not global gray matter atrophy occurs in multiple sclerosis. Our aim was to identify specific areas of gray matter volume changes and explore the relationship between atrophy and clinical motor outcomes. Nine patients with relapsing remitting MS and 9 matched healthy controls were recruited. The Multiple Sclerosis Functional Composite was administered. For MR acquisitions, a GE- Genesis- Signa, 1.5T MR system, was used. A voxel-based morphometry (VBM), subcortical structures segmentation (FIRST) and volumetric (SIENAx) FSL tools were used in the study. Group comparison showed atrophy for several gray matter regions. The most important volume reductions were found for subcortical deep gray matter areas. Correlations with clinical scores were checked and specific gray matter areas showed significant volume reductions associated with motor scores (9-hole peg time and 25-feet walk time) and EDSS (Expanded Disability Status Scale). We performed a voxelwise analysis of gray matter changes in MS and found a more prominent atrophy for the subcortical structures than for cortical gray matter. Using an additional analysis (FIRST and SIENAx segmentation/volumetry) we were able to confirm the VBM results and to quantify the degree of atrophy in specific structures. Specific gray matter regions which volume reductions correlate with 25-feet walk, 9-hole peg times and EDSS suggest that 25-feet walk time is the best predictor of disease progression in terms of gray matter reduction.

  15. Diffusion abnormality maps in demyelinating disease: Correlations with clinical scores

    International Nuclear Information System (INIS)

    Onu, Mihaela; Roceanu, Adina; Sboto-Frankenstein, Uta; Bendic, Robert; Tarta, Eugen; Preoteasa, Florentin; Bajenaru, Ovidiu

    2012-01-01

    Background and purpose: Magnetic resonance imaging (MRI) has been explored as a noninvasive tool to assess pathology in multiple sclerosis (MS) patients. However, the correlation between classical MRI measures and physical disability is modest in MS. The diffusion tensor imaging (DTI) MRI technique holds particular promise in this regard. The present study shows brain regions where FA and individual diffusivities abnormalities are present and check their correlations with physical disability clinical scores. Methods: Eight patients and 12 matched healthy controls were recruited. The Multiple Sclerosis Functional Composite was administered. For MR-DTI acquisitions, a Genesis Signa 1.5T MR system, an EP/SE scanning sequence, 25 gradient directions were used. Results: Tract Based Spatial Statistics (TBSS) group comparisons showed reduced FA and increased individual diffusivities in several brain regions in patients. Significant correlations were found between FA and: EDSS, 9-HPT(NON)DOM and 25FW score; between λ 2 and: P100 (r and l), 9-HPT(NON)DOM and 25FW; between λ 3 and: 9-HPT(NON)DOM and 25FW score. Conclusions: Fractional anisotropy and individual radial diffusivities proved to be important markers of motor disabilities in MS patients when the disease duration mean and the disability scores values range are relatively high.

  16. Physiological Dynamics in Demyelinating Diseases: Unraveling Complex Relationships through Computer Modeling

    Directory of Open Access Journals (Sweden)

    Jay S. Coggan

    2015-09-01

    Full Text Available Despite intense research, few treatments are available for most neurological disorders. Demyelinating diseases are no exception. This is perhaps not surprising considering the multifactorial nature of these diseases, which involve complex interactions between immune system cells, glia and neurons. In the case of multiple sclerosis, for example, there is no unanimity among researchers about the cause or even which system or cell type could be ground zero. This situation precludes the development and strategic application of mechanism-based therapies. We will discuss how computational modeling applied to questions at different biological levels can help link together disparate observations and decipher complex mechanisms whose solutions are not amenable to simple reductionism. By making testable predictions and revealing critical gaps in existing knowledge, such models can help direct research and will provide a rigorous framework in which to integrate new data as they are collected. Nowadays, there is no shortage of data; the challenge is to make sense of it all. In that respect, computational modeling is an invaluable tool that could, ultimately, transform how we understand, diagnose, and treat demyelinating diseases.

  17. Tumefactive demyelinating lesions

    International Nuclear Information System (INIS)

    Dagher, A.P.; Smirniotopoulos, J.; Armed Forces Inst. of Pathology, Washington, DC

    1996-01-01

    We studied 21 cases of pathologically confirmed tumefactive demyelinating lesions and reviewed the spectrum of tumefactive demyelinating lesions in the literature. Radiological features and clinical data were reviewed to characterize the lesions as consistent with a known demyelinating disease, most notably multiple sclerosis. Atypical clinical or radiological features (other than tumefaction) were noted. Most lesions were part of a clinical and/or radiological picture consistent with multiple sclerosis. No case strongly suggestive of variants or related diseases, such as Schilder's disease or Balo's concentric sclerosis, were found. There was one case suggestive of acute disseminated encephalomyelitis. Features which help distinguish the lesions from tumour are discussed. (orig.)

  18. Tumefactive demyelinating lesions

    Energy Technology Data Exchange (ETDEWEB)

    Dagher, A.P. [Thomas Jefferson Univ. Hospital, Philadelphia, PA (United States). Div. of Neuroradiology; Smirniotopoulos, J. [Thomas Jefferson Univ. Hospital, Philadelphia, PA (United States). Div. of Neuroradiology]|[Armed Forces Inst. of Pathology, Washington, DC (United States). Dept. of Radiological Pathology

    1996-08-01

    We studied 21 cases of pathologically confirmed tumefactive demyelinating lesions and reviewed the spectrum of tumefactive demyelinating lesions in the literature. Radiological features and clinical data were reviewed to characterize the lesions as consistent with a known demyelinating disease, most notably multiple sclerosis. Atypical clinical or radiological features (other than tumefaction) were noted. Most lesions were part of a clinical and/or radiological picture consistent with multiple sclerosis. No case strongly suggestive of variants or related diseases, such as Schilder`s disease or Balo`s concentric sclerosis, were found. There was one case suggestive of acute disseminated encephalomyelitis. Features which help distinguish the lesions from tumour are discussed. (orig.)

  19. Tolerance of intravenous methylprednisolone for relapse treatment in demyelinating CNS disease.

    Science.gov (United States)

    Lienert, Carmen; Schawalder, Gabriela; Findling, Oliver; Kamm, Christian P; Humpert, Sebastian; Mugglin, Anne; Mathis, Johannes; Sturzenegger, Mathias; Mattle, Heinrich P

    2013-05-28

    In Switzerland, the first course of intravenous steroids for treatment of episodes of demyelinating CNS disease is usually administered in an inpatient setting. We prospectively evaluated short term tolerance of treatment with special emphasis on sleep quality. Patients with a first event of presumed demyelinating disease (CIS), multiple sclerosis relapses (MS) or sub-acute disease progression were treated with a 5-day regimen of intravenous methylprednisolone (IVMP) in our inpatient clinic. Patients' experience was documented by self-report questionnaires including a standardised depression scale (ADSL). Laboratory tests were performed on a routine basis. Fasting glucose, blood pressure and pulse were measured before every infusion. Activity and sleep patterns were analysed by wrist actigraphs during the 5 day infusion period and at follow-up after 1-2 months. A total of 66 patients participated in the study. Of these, 55 were steroid treatment naïve, and 11 patients, who had received intravenous steroid relapse treatment before, were admitted because of disabling symptoms. Mood disturbances were reported before steroid treatment, however significantly less often at the end of the steroid pulse and during follow-up. Sleep efficiency as measured by wrist actimetry was high before, during and after steroid treatment. Therapy was well tolerated without severe side effects in CIS and MS patients. Sleep efficiency was not disturbed. In conclusion there are no obstacles to change from an inpatient to an outpatient setting for the steroid treatment of relapses in MS and CIS, but rare psychotic reactions to steroid treatment are not predictable.

  20. Demyelinating disease and anti-N-methyl-D-aspartate receptor immunoglobulin G antibodies: a case report.

    Science.gov (United States)

    Waschbisch, Anne; Kallmünzer, Bernd; Schwab, Stefan; Gölitz, Philipp; Vincent, Angela; Lee, De-Hyung; Linker, Ralf A

    2014-12-23

    Anti-N-methyl-D-aspartate receptor immunoglobulin G antibodies directed against the GluN1 subunit are considered highly specific for anti-N-methyl-D-aspartate receptor encephalitis, a severe clinical syndrome characterized by seizures, psychiatric symptoms, orofacial dyskinesia and autonomic dysfunction. Here we report a 33 year old Caucasian male patient with clinically definite multiple sclerosis who was found to be positive for anti-N-methyl-D-aspartate receptor antibodies. Rituximab therapy was initiated. On the 18 months follow-up visit the patient was found to be clinically stable, without typical signs of anti-N-methyl-D-aspartate receptor encephalitis. Our findings add to the growing evidence for a possible association between anti-N-methyl-D-aspartate receptor encephalitis and demyelinating diseases.

  1. Targeting insulin-like growth factor 1 leads to amelioration of inflammatory demyelinating disease.

    Directory of Open Access Journals (Sweden)

    Matthew F Cusick

    Full Text Available In patients with multiple sclerosis (MS and in mice with experimental autoimmune encephalomyelitis (EAE, proliferating autoreactive T cells play an important role in the pathogenesis of the disease. Due to the importance of these myelin-specific T cells, these cells have been therapeutic targets in a variety of treatments. Previously we found that Lenaldekar (LDK, a novel small molecule, could inhibit exacerbations in a preclinical model of MS when given at the start of an EAE exacerbation. In those studies, we found that LDK could inhibit human T cell recall responses and murine myelin responses in vitro. In these new studies, we found that LDK could inhibit myelin specific T cell responses through the insulin-like growth factor-1 receptor (IGF-1R pathway. Alteration of this pathway led to marked reduction in T cell proliferation and expansion. Blocking this pathway could account for the observed decreases in clinical signs and inflammatory demyelinating disease, which was accompanied by axonal preservation. Our data indicate that IGF-1R could be a potential target for new therapies for the treatment of autoimmune diseases where autoreactive T cell expansion is a requisite for disease.

  2. Charcot-Marie-Tooth disease type 2 caused by homozygous MME gene mutation superimposed by chronic inflammatory demyelinating polyneuropathy.

    Science.gov (United States)

    Fujisawa, Miwako; Sano, Yasuteru; Omoto, Masatoshi; Ogasawara, Jyun-Ichi; Koga, Michiaki; Takashima, Hiroshi; Kanda, Takashi

    2017-09-30

    We report a 59-year-old Japanese male who developed gradually worsening weakness and numbness of distal four extremities since age 50. His parents were first cousins, and blood and cerebral spinal examinations were unremarkable. Homozygous mutation of MME gene was detected and thus he was diagnosed as autosomal-recessive Charcot-Marie-Tooth disease 2T (AR-CMT2T); however, electrophysiological examinations revealed scattered demyelinative changes including elongated terminal latency in several peripheral nerve trunks. Sural nerve biopsy showed endoneurial edema and a lot of thinly myelinated nerve fibers with uneven distribution of remnant myelinated fibers within and between fascicles. Immunoglobulin treatment was initiated considering the possibility of superimposed inflammation and demyelination, and immediate clinical as well as electrophysiological improvements were noted. Our findings indicate that AR-CMT2T caused by MME mutation predisposes to a superimposed inflammatory demyelinating neuropathy. This is the first report which documented the co-existence of CMT2 and chronic inflammatory demyelinating polyneuropathy (CIDP); however, in the peripheral nervous system, neprilysin, a product of MME gene, is more abundant in myelin sheath than in axonal component. The fragility of myelin sheath due to mutated neprilysin may trigger the detrimental immune response against peripheral myelin in this patient.

  3. Demyelinizing Neurological Disease after Treatment with Tumor Necrosis Factor-α Antagonists

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    Claudia Bruè

    2016-07-01

    Full Text Available Purpose: Demyelinizing neurological disease is a rare complication after treatment with tumor necrosis factor (TNFα antagonists. We report on a case of multiple sclerosis after TNFα antagonist treatment and discuss its differential diagnosis. Methods: This is an observational case study. Results: A 48-year-old male was referred to Ophthalmology in January 2015 for an absolute scotoma in the superior quadrant of the visual field in his right eye. Visual acuity was 20/50 in the right eye and 20/20 in the left. Fundus examination was unremarkable bilaterally. Spectral domain optical coherence tomography revealed a normal macular retina structure. Visual field examination revealed a superior hemianopsia in the right eye. Head magnetic resonance imaging showed findings compatible with optic neuritis. The visual evoked potentials confirmed the presence of optic neuritis. The patient had been under therapy with adalimumab since January 2014, for Crohn’s disease. Suspension of adalimumab was recommended, and it was substituted with tapered deltacortene, from 1 mg/kg/day. After 1 month, the scotoma was resolved completely. Conclusions: TNFα antagonists can provide benefit to patients with inflammatory autoimmune diseases. However, they can also be associated with severe adverse effects. Therefore, adequate attention should be paid to neurological abnormalities in patients treated with TNFα antagonists.

  4. The effect of glia-glia interactions on oligodendrocyte precursor cell biology during development and in demyelinating diseases

    OpenAIRE

    Clemente, Diego; Ortega, M.C.; Melero-Jerez, Carolina; Castro Soubriet, Fernando de

    2013-01-01

    Oligodendrocyte precursor cells (OPCs) originate in specific areas of the developing central nervous system (CNS). Once generated, they migrate towards their destinations where they differentiate into mature oligodendrocytes. In the adult, 5–8% of all cells in the CNS are OPCs, cells that retain the capacity to proliferate, migrate, and differentiate into oligodendrocytes. Indeed, these endogenous OPCs react to damage in demyelinating diseases, like multiple sclerosis (MS), representing a key...

  5. Constitutive expression of a costimulatory ligand on antigen-presenting cells in the nervous system drives demyelinating disease

    DEFF Research Database (Denmark)

    Zehntner, Simone P; Brisebois, Marcel; Tran, Elise

    2003-01-01

    that transgenic mice constitutively expressing the costimulatory ligand B7.2/CD86 on microglia in the central nervous system (CNS) and on related cells in the proximal peripheral nervous tissue spontaneously develop autoimmune demyelinating disease. Disease-affected nervous tissue in transgenic mice showed...... recipients but not into non-transgenic recipients. These data provide evidence that B7/CD28 interactions within the nervous tissue are critical determinants of disease development. Our findings have important implications for understanding the etiology of nervous system autoimmune diseases such as multiple...

  6. Oligodendroglia in cortical multiple sclerosis lesions decrease with disease progression, but regenerate after repeated experimental demyelination.

    Science.gov (United States)

    Rodriguez, Enrique Garea; Wegner, Christiane; Kreutzfeldt, Mario; Neid, Katharina; Thal, Dietmar R; Jürgens, Tanja; Brück, Wolfgang; Stadelmann, Christine; Merkler, Doron

    2014-08-01

    Cerebral cortex shows a high endogenous propensity for remyelination. Yet, widespread subpial cortical demyelination (SCD) is a common feature in progressive multiple sclerosis (MS) and can already be found in early MS. In the present study, we compared oligodendroglial loss in SCD in early and chronic MS. Furthermore, we addressed in an experimental model whether repeated episodes of inflammatory SCD could alter oligodendroglial repopulation and subsequently lead to persistently demyelinated cortical lesions. NogoA(+) mature oligodendrocytes and Olig2(+) oligodendrocyte precursor cells were examined in SCD in patients with early and chronic MS, normal-appearing MS cortex, and control cortex as well as in the rat model of repeated targeted cortical experimental autoimmune encephalomyelitis (EAE). NogoA(+) and Olig2(+) cells were significantly reduced in SCD in patients with chronic, but not early MS. Repeated induction of SCD in rats resulted only in a transient loss of NogoA(+), but not Olig2(+) cells during the demyelination phase. This phase was followed by complete oligodendroglial repopulation and remyelination, even after four episodes of demyelination. Our data indicate efficient oligodendroglial repopulation in subpial cortical lesions in rats after repeated SCD that was similar to early, but not chronic MS cases. Accordingly, four cycles of experimental de- and remyelination were not sufficient to induce sustained remyelination failure as found in chronic cortical MS lesions. This suggests that alternative mechanisms contribute to oligodendrocyte depletion in chronic cortical demyelination in MS.

  7. Consensus Statement on medication use in multiple sclerosis by the Spanish Society of Neurology's study group for demyelinating diseases.

    Science.gov (United States)

    García-Merino, A; Fernández, O; Montalbán, X; de Andrés, C; Oreja-Guevara, C; Rodríguez-Antigüedad, A; Arbizu, T

    2013-01-01

    Treatments for multiple sclerosis therapy are rapidly evolving. It is believed that new drugs will be approved in the near future, thereby changing current indications for treatment. In this context, the Spanish Society of Neurology's study group on demyelinating diseases, which evaluates medication use in MS, has decided to draw up a consensus statement on the current indications and guidelines for multiple sclerosis treatment. Copyright © 2013 Sociedad Española de Neurología. Published by Elsevier Espana. All rights reserved.

  8. Demyelinating disease in patients with myasthenia gravis Doenças desmielinizantes em pacientes com miastenia gravis

    Directory of Open Access Journals (Sweden)

    Denis Bernardi Bichuetti

    2008-03-01

    Full Text Available Myasthenia gravis (MG is an autoimmune disease characterized by fluctuating muscle weakness, caused by impaired neuromuscular transmission. Patients with MG can present other autoimmune diseases in association, commonly hypo or hyperthyroidism. The association of MG to demyelinating disease is rare and has been described before. We report on three Brazilian patients with MG that presented distinct demyelinating diseases, two monophasic and one recurrent neuromyelitis optica, several years after the diagnosis of MG, and discuss their clinical courses.Miastenia gravis (MG é doença autoimune caracterizada por episódios de fraqueza muscular alternados com melhora, causada por bloqueio da junção neuromuscular. Pacientes com MG podem apresentar outras doenças autoimunes, comumente hipo ou hipertiroidismo, e a associação de MG com doenças desmielinizantes é raramente descrita. Relatamos três pacientes brasileiros com MG que desenvolveram doenças desmielinizantes, dois monofásicos e um neuromielite óptica recorrente, vários anos após o diagnóstico de MG e discutimos seus cursos clínicos.

  9. Disease Type- and Status-Specific Alteration of CSF Metabolome Coordinated with Clinical Parameters in Inflammatory Demyelinating Diseases of CNS.

    Directory of Open Access Journals (Sweden)

    Soo Jin Park

    Full Text Available Central nervous system (CNS inflammatory demyelinating diseases (IDDs are a group of disorders with different aetiologies, characterized by inflammatory lesions. These disorders include multiple sclerosis (MS, neuromyelitis optica spectrum disorder (NMOSD, and idiopathic transverse myelitis (ITM. Differential diagnosis of the CNS IDDs still remains challenging due to frequent overlap of clinical and radiological manifestation, leading to increased demands for new biomarker discovery. Since cerebrospinal fluid (CSF metabolites may reflect the status of CNS tissues and provide an interfacial linkage between blood and CNS tissues, we explored multi-component biomarker for different IDDs from CSF samples using gas chromatography mass spectrometry-based metabolite profiling coupled to multiplex bioinformatics approach. We successfully constructed the single model with multiple metabolite variables in coordinated regression with clinical characteristics, expanded disability status scale, oligoclonal bands, and protein levels. The multi-composite biomarker simultaneously discriminated four different immune statuses (a total of 145 samples; 54 MS, 49 NMOSD, 30 ITM, and 12 normal controls. Furthermore, systematic characterization of transitional metabolic modulation identified relapse-associated metabolites and proposed insights into the disease network underlying type-specific metabolic dysfunctionality. The comparative analysis revealed the lipids, 1-monopalmitin and 1-monostearin were common indicative for MS, NMOSD, and ITM whereas fatty acids were specific for the relapse identified in all types of IDDs.

  10. Tangier′s disease: An uncommon cause of facial weakness and non-length dependent demyelinating neuropathy

    Directory of Open Access Journals (Sweden)

    Madhu Nagappa

    2016-01-01

    Full Text Available Tangier disease is an autosomal recessive disorder characterized by an abnormal accumulation of cholesterol esters in various organs secondary to adenotriphosphate binding cassette transporter A-1 (ABCA-1 transporter deficiency and disrupted reverse cholesterol transport. It causes neuropathy in half of the affected individuals. We present the clinical, electrophysiological, and histopathological findings in a middle aged gentleman of Tangier disease who was initially misdiagnosed leprosy and treated with antileprosy drugs. The presence of a demyelinating neuropathy on electrophysiology in a patient with predominant upper limb involvement and facial diplegia should raise the suspicion of Tangier disease. The characteristic lipid profile of Tangier disease was noted in this patient viz. extremely low high density lipoprotein (HDL, elevated triglyceride (TG, and reduced apolipoprotein A1. Estimation of serum lipids should form a part of routine evaluation in order to avoid misdiagnosis.

  11. Correlation between spinal cord MRI and clinical features in patients with demyelinating disease

    International Nuclear Information System (INIS)

    Papadopoulos, A.; Gatzonis, S.; Gouliamos, A.; Trakadas, S.; Kalovidouris, A.; Sgouropoulos, P.; Vlachos, L.; Papavasiliou, C.

    1994-01-01

    Localisation of spinal cord lesions by MRI was correlated with neurological symptoms and signs in 16 patients with clinical and laboratory evidence of multiple sclerosis. There was good correspondence between spinal cord lesions and motor tract signs. On the other hand, superficial or deep sensory disturbances correlated with spinal cord lesions in only about a quarter of the patients. MRI of the spinal cord appeared to explain the myelopathy in 11 patients, while in 3 there was strong clinical evidence of more extensive demyelinating lesions. In 7 of the 16 patients MRI of the brain was normal. (orig.)

  12. The effect of glia-glia interactions on oligodendrocyte precursor cell biology during development and in demyelinating diseases

    Directory of Open Access Journals (Sweden)

    Diego eClemente

    2013-12-01

    Full Text Available Oligodendrocyte precursor cells (OPCs originate in specific areas of the developing central nervous system (CNS. Once generated, they migrate towards their destinations where they differentiate into mature oligodendrocytes. In the adult, 5-8% of all cells in the CNS are OPCs, cells that retain the capacity to proliferate, migrate and differentiate into oligodendrocytes. Indeed, these endogenous OPCs react to damage in demyelinating diseases, like multiple sclerosis (MS, representing a key element in spontaneous remyelination. In the present work, we review the specific interactions between OPCs and other glial cells (astrocytes, microglia during CNS development and in the pathological scenario of MS. We focus on: i the role of astrocytes in maintaining the homeostasis and spatial distribution of different secreted cues that determine OPC proliferation, migration and differentiation during CNS development; ii the role of microglia and astrocytes in the redistribution of iron, which is crucial for myelin synthesis during CNS development and for myelin repair in MS; iii how microglia secrete different molecules, e.g. growth factors, that favor the recruitment of OPCs in acute phases of MS lesions; and iv how astrocytes modify the extracellular matrix in MS lesions, affecting the ability of OPCs to attempt spontaneous remyelination. Together, these issues demonstrate how both astroglia and microglia influence OPCs in physiological and pathological situations, reinforcing the concept that both development and neural repair are complex and global phenomena. Understanding the molecular and cellular mechanisms that control OPC survival, proliferation, migration and differentiation during development, as well as in the mature CNS, may open new opportunities in the search for reparative therapies in demyelinating diseases like MS.

  13. Clinical features in very early-onset demyelinating disease with anti-MOG antibody.

    Science.gov (United States)

    Nishiyama, Masahiro; Nagase, Hiroaki; Matsumoto, Masaaki; Tomioka, Kazumi; Awano, Hiroyuki; Tanaka, Tsukasa; Toyoshima, Daisaku; Fujita, Kyoko; Maruyama, Azusa; Oyazato, Yoshinobu; Saeki, Keisuke; Shiraishi, Kazuhiro; Takada, Satoshi; Kaneko, Kimihiko; Takahashi, Toshiyuki; Nakashima, Ichiro; Iijima, Kazumoto

    2017-10-01

    The clinical features of patients with very early-onset acquired demyelinating syndrome (ADS) with the anti-myelin oligodendrocyte glycoprotein (MOG) antibody are unknown. We investigated the clinical characteristics and described detailed treatment of weekly intramuscular interferon β-1a (IFNβ-1a) in children aged <4years with ADS and the anti-MOG antibody. We conducted a retrospective chart review of patients with anti-MOG positivity who were diagnosed as having multiple sclerosis (MS) at <4years of age. Subjects comprised 2 boys and 2 girls. Initial symptoms included ataxia, facial paresis, status epilepticus, and encephalopathy. Abnormal lesions on magnetic resonance imaging scans were often detected in the brainstem and cerebellum as well as the cerebrum. All patients started receiving IFNβ-1a at age 3.1-3.5years. The initial doses ranged from 3 to 6μg, which were 1/10-1/5 doses, respectively, for adults. During 0.6-4.3years of IFNβ-1a administration, all patients had flu-like symptoms, and 1 patient had an increased liver enzyme level. Although 1 patient discontinued IFNβ-1a therapy because of frequent relapses, no patient discontinued therapy due to severe adverse events. This case series adds novel information regarding the clinical features of children <4years old with ADS and the anti-MOG antibody. Copyright © 2017 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

  14. Kikuchi-fujimoto disease, the masquerading menace: A rare case report

    Directory of Open Access Journals (Sweden)

    Rohit Kataria

    2016-01-01

    Full Text Available Kikuchi-Fujimoto disease (KFD or histiocytic necrotizing lymphadenitis is a rare, benign, self-limiting disease with unknown etiology characterized by regional lymphadenopathy. A 30-year-old female presented with fever, weakness, multiple joint pain, oral ulcers, erythematous facial rashes, hemorrhagic crusting on both lips, and cervical lymphadenopathy of 2-month duration. Clinically, the disease was mimicking systemic lupus erythematosus, but immunofluorescence was negative for it. Lymph node biopsy suggested a diagnosis of KFD.

  15. Early Disseminated Lyme Disease Masquerading as Mononucleosis: A Case Report.

    Science.gov (United States)

    Tumminello, Richard; Glaspey, Lindsey; Bhamidipati, Anita; Sheehan, Patrick; Patel, Sundip

    2017-12-01

    Disseminated Lyme disease can be difficult to diagnose, as it begins with nonspecific signs and symptoms, which, if not treated correctly, can lead to atrioventricular conduction blocks and meningitis. In addition, the diagnosis can be further complicated by potentially false-positive test results. We report a case of early-disseminated Lyme disease presenting with Borrelia meningitis and concomitant Lyme carditis, which was misdiagnosed as mononucleosis. A young, previously healthy patient had been hiking in the woods of upstate New York and 4 weeks later developed fever, night sweats, and myalgias. He was diagnosed with mononucleosis via a positive rapid heterophile agglutination antibody test to the Epstein-Barr virus at a walk-in clinic and was started on medications, but then subsequently developed left hip pain, a facial droop, and a very long first-degree atrioventricular conduction block. He went to the Emergency Department, where he had testing that confirmed disseminated Lyme disease. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: This case highlights the difficulty in early diagnosis of disseminated Lyme disease and how a potentially false-positive laboratory test can lead to the complications of Borrelia meningitis and Lyme carditis in untreated young healthy patients. Emergency physicians need to consider Lyme disease in patients with nonspecific signs and symptoms, especially if they have been outdoors for prolonged periods of time in Lyme-endemic areas. Copyright © 2017 Elsevier Inc. All rights reserved.

  16. Non-hogkins lymphoma 'masquerading' as pott's disease in a 13 ...

    African Journals Online (AJOL)

    Lymphomas are malignant neoplasms of the lymphoid lineage which are broadly classified as either Hodgkin disease or as non-Hodgkin lymphoma (NHL). Burkitt lymphoma, a variety of NHL, is most common in sub-Saharan Africa, where it accounts for approximately one half of childhood cancers. A case of paravertebral ...

  17. Masquerading acidosis after cardiopulmonary bypass: a case of propionic acidemia and congenital heart disease.

    Science.gov (United States)

    Palermo, Robert A; Monge, Michael C; Charrow, Joel; Costello, John M; Epting, Conrad L

    2015-04-01

    We report the case of a child with both propionic acidemia and cyanotic congenital heart disease. The presence of an underlying inborn error of metabolism confounded the management of this patient in the postoperative period, resulting in therapeutic misdirection until the true etiology of hyperlactemia was recognized. © The Author(s) 2014.

  18. Early Motor Unit Disease Masquerading as Psychogenic Breathy Dysphonia: A Clinical Case Presentation

    Science.gov (United States)

    Aronson, Arnold E.

    1971-01-01

    Presented is a study of a 20-year-old girl with mild, breathy dysphonia, previously diagnosed as psychogenic. In actuality, her voice change was a sign of early myasthenia gravis. It is pointed out that voice changes can be a first and only sign of early neurologic disease. (Author/KW)

  19. Creutzfeldt-Jakob disease: A great masquerade in neurology, a rare case report from South India

    Directory of Open Access Journals (Sweden)

    Sivaprakash Varadan

    2015-01-01

    Full Text Available Creutzfeldt-Jakob disease (CJD is a rare, fatal neurodegenerative disease caused by an infectious protein called prion and is characterized by spongiform changes, neuronal loss, reactive astrocytic proliferation, and accumulation of pathologic cellular protein. Clinical presentation of CJD is characterized by rapidly progressive dementia, neurologic symptoms and visual impairment, and the development of akinetic mutism, which can mimic many neurological conditions. The diagnosis is based on clinical presentation, electroencephalogram, and typical cerebrospinal fluid and magnetic resonance imaging (MRI findings. Literature on the incidence and prevalence of CJD is lacking in South India. We report the case of a 57-year-old woman with progressive dementia and typical neurologic symptoms, myoclonic jerks, and MRI findings of CJD. This case highlights the need for a high index of suspicion to diagnose CJD.

  20. Rapidly progressive hip disease masquerading as Gorham′s syndrome of the femoral head

    Directory of Open Access Journals (Sweden)

    Atul Mahajan

    2016-01-01

    Full Text Available Rapidly destructive hip disease (RDHD is a rare syndrome of unknown etiology, resulting in rapid deterioration of both the femoral and acetabular aspects of the hip joint with disappearance of the femoral head. Differential diagnosis should include those conditions known to potentially lead to rapid hip destruction, such as septic arthritis, metabolic bone diseases, autoimmune inflammatory arthritis, malignancy, and classical osteonecrosis. Sequential X-rays in patients with fast worsening of hip symptoms and a high degree of clinical suspicion seem mandatory to avoid extensive joint destruction and facilitate better arthroplasty outcomes in these patients. Because of the degree of joint deformity and the patient′s level of disability, the typical treatment of rapidly destructive arthropathy is total hip arthroplasty. In this report, we present a clinical case of left RDHD offering a useful review for the diagnosis and management of this condition.

  1. Re-Emergent Tremor of Parkinson's Disease Masquerading as Essential Tremor

    Directory of Open Access Journals (Sweden)

    Sarah Morgan

    2016-03-01

    Full Text Available Background: The re-emergent tremor of Parkinson’s disease (PD is generally recognized as a postural tremor. Phenomenology Shown: A PD patient with a re-emergent tremor occurring during a task (spiral drawing, which on the surface produced a tremor that resembled that of essential tremor (ET. Educational Value: Researchers and clinicians should be aware of features of this re-emergent tremor to help distinguish it from that of ET.

  2. Demyelinating Disease following Anti-TNFa Treatment: A Causal or Coincidental Association? Report of Four Cases and Review of the Literature

    Directory of Open Access Journals (Sweden)

    E. Andreadou

    2013-01-01

    Full Text Available Tumor necrosis factor antagonists (anti-TNFa are an established therapeutic option for several autoimmune and inflammatory bowel diseases. Despite their clinical effectiveness, neurological adverse events have been reported and literature data suggest a potential role of anti-TNFa in the induction of demyelination of the CNS. We present four patients treated with anti-TNFa who developed symptoms suggestive of CNS demyelination. The first patient, a 17-year-old male who received etanercept for psoriatic arthritis for eight months, presented with dysesthesias up to T4 level. The second patient, a 30-year-old male treated with adalimumab for three years due to ankylosing spondylitis, presented with right unilateral tinnitus. The third case, a 47-year-old female, received etanercept for four years because of psoriatic arthritis and developed persistent headache and left-sided face and head numbness. Finally, the fourth patient, a 57-years-old female treated with etanercept for six years due to ankylosing spondylitis, presented with difficulty in speech, swallowing, and ptosis of the right corner of the mouth. In all cases, brain MRI showed lesions suggestive of demyelination, while positive oligoclonal bands were detected in the CSF. Anti-TNFa treatments were discontinued and patients showed clinical improvement with pulsed intravenous corticosteroid therapy. CNS demyelination following anti-TNFa treatment represents a relatively rare but potential serious complication. Close follow-up and MRI monitoring of these patients is mandatory to elucidate whether the clinical manifestations represent adverse events occurring during anti-TNFa therapy or a first demyelinating episode.

  3. C1 Inhibitor Deficiency and Angioedema of the Small Intestine Masquerading as Crohn’s Disease

    Directory of Open Access Journals (Sweden)

    Kelly W Burak

    2000-01-01

    Full Text Available A case of C1 inhibitor deficiency presenting as localized edema of the small intestine is described. A 16-year-old, previously healthy woman presented with recurrent attacks of abdominal pain and vomiting following minor abdominal trauma. Investigations including computed tomography scan and barium studies confirmed localized edema of the jejunum. At laparoscopy, Crohn’s disease was suspected; however, a subsequent enteroscopy was normal. Complement levels revealed a low C4 level, and C1 inhibitor deficiency was later confirmed. Attacks of abdominal pain began after starting oral contraceptives and have not returned since stopping the birth control pill. This rare cause of abdominal pain is examined, and C1 inhibitor deficiency and angioedema are reviewed.

  4. Amnesia in Frontotemporal Dementia with Amyotrophic Lateral Sclerosis, Masquerading Alzheimer’s Disease

    Directory of Open Access Journals (Sweden)

    A. Yamanami-Irioka

    2011-10-01

    Full Text Available A 68-year-old man with a clinical diagnosis of Alzheimer’s disease (AD later developed amyotrophic lateral sclerosis (ALS, which was confirmed at autopsy at age 72 years. Because neuronal loss and AD-type pathologies (Braak stage II for neurofibrillary tangles were scant, TDP-43-positive intracytoplasmic inclusions in hippocampal dentate granular cells and in neurons in the subiculum and amygdala, even though small in amount, may represent the earliest lesions of ALS-related dementia and could be the cause of dementia in this patient. Although the persistent elevation of creatine kinase from the onset could be a pointer to the presence of motor involvement, more accurate characterization of dementia, which may differentiate ALS-related dementia and AD, is necessary.

  5. Autoimmune Demyelinating Polyneuropathy as a Manifestation of Chronic Graft-versus-Host Disease after Adult Cord Blood Transplantation in a Patient with Chronic Lymphocytic Leukemia

    Directory of Open Access Journals (Sweden)

    Fredrick Hogan

    2014-01-01

    Full Text Available Immune mediated demyelinating disease after allogeneic stem cell transplantation is a rare entity with unclear etiology. Acute inflammatory demyelinating polyneuropathy (AIDP has been reported after related and adult unrelated allogeneic stem cell transplantation but no such case has been reported after unrelated cord blood transplantation. We hereby present the first case of AIDP after double umbilical cord blood transplantation (DUCBT. A 55-year-old man with chronic lymphocytic leukemia (CLL received a cord blood transplant for relapsed refractory disease with high risk cytogenetics. On day 221, patient presented with skin rash, tingling in both lower extremites, and ascending paralysis that progressed rapidly over the course of 2 days. The workup resulted in a diagnosis of AIDP and administration of intravenous immunoglobulins plus steroids was initiated. Motor and sensory powers were fully recovered and his chronic GVHD was managed for several months with single agent sirolimus.

  6. Epistaxis in end stage liver disease masquerading as severe upper gastrointestinal hemorrhage.

    Science.gov (United States)

    Camus, Marine; Jensen, Dennis M; Matthews, Jason D; Ohning, Gordon V; Kovacs, Thomas O; Jutabha, Rome; Ghassemi, Kevin A; Machicado, Gustavo A; Dulai, Gareth S

    2014-10-14

    To describe the prevalence, diagnosis, treatment, and outcomes of end stage liver disease (ESLD) patients with severe epistaxis thought to be severe upper gastrointestinal hemorrhage (UGIH). This observational single center study included all consecutive patients with ESLD and epistaxis identified from consecutive subjects hospitalized with suspected UGIH and prospectively enrolled in our databases of severe UGIH between 1998 and 2011. A total of 1249 patients were registered for severe UGIH in the data basis, 461 (36.9%) were cirrhotics. Epistaxis rather than UGIH was the bleeding source in 20 patients. All patients had severe coagulopathy. Epistaxis was initially controlled in all cases. Fifteen (75%) subjects required posterior nasal packing and 2 (10%) embolization in addition to correction of coagulopathy. Five (25%) patients died in the hospital, 12 (60%) received orthotopic liver transplantation (OLT), and 3 (15%) were discharged without OLT. The mortality rate was 63% in patients without OLT. Severe epistaxis in patients with ESLD is (1) a diagnosis of exclusion that requires upper endoscopy to exclude severe UGIH; and (2) associated with a high mortality rate in patients not receiving OLT.

  7. TLR3 signaling is either protective or pathogenic for the development of Theiler's virus-induced demyelinating disease depending on the time of viral infection

    Directory of Open Access Journals (Sweden)

    Jin Young-Hee

    2011-12-01

    Full Text Available Abstract Background We have previously shown that toll-like receptor 3 (TLR3-mediated signaling plays an important role in the induction of innate cytokine responses to Theiler's murine encephalomyelitis virus (TMEV infection. In addition, cytokine levels produced after TMEV infection are significantly higher in the glial cells of susceptible SJL mice compared to those of resistant C57BL/6 mice. However, it is not known whether TLR3-mediated signaling plays a protective or pathogenic role in the development of demyelinating disease. Methods SJL/J and B6;129S-Tlr3tm1Flv/J (TLR3KO-B6 mice, and TLR3KO-SJL mice that TLR3KO-B6 mice were backcrossed to SJL/J mice for 6 generations were infected with Theiler's murine encephalomyelitis virus (2 × 105 PFU with or without treatment with 50 μg of poly IC. Cytokine production and immune responses in the CNS and periphery of infected mice were analyzed. Results We investigated the role of TLR3-mediated signaling in the protection and pathogenesis of TMEV-induced demyelinating disease. TLR3KO-B6 mice did not develop demyelinating disease although they displayed elevated viral loads in the CNS. However, TLR3KO-SJL mice displayed increased viral loads and cellular infiltration in the CNS, accompanied by exacerbated development of demyelinating disease, compared to the normal littermate mice. Late, but not early, anti-viral CD4+ and CD8+ T cell responses in the CNS were compromised in TLR3KO-SJL mice. However, activation of TLR3 with poly IC prior to viral infection also exacerbated disease development, whereas such activation after viral infection restrained disease development. Activation of TLR3 signaling prior to viral infection hindered the induction of protective IFN-γ-producing CD4+ and CD8+ T cell populations. In contrast, activation of these signals after viral infection improved the induction of IFN-γ-producing CD4+ and CD8+ T cells. In addition, poly IC-pretreated mice displayed elevated PDL-1 and

  8. Role of Th1 and Th2 cells in autoimmune demyelinating disease

    NARCIS (Netherlands)

    Nagelkerken, L.

    1998-01-01

    Evidence is accumulating that Th1 cells play an important role in the development of multiple sclerosis (MS) and experimental allergic encephalomyelitis (EAE), whereas Th2 cells contribute to recovery from disease. A maj or determinant in the development of Th1 and Th2 cells is the type of

  9. Pathological differences in acute inflammatory demyelinating diseases of the central nervous system.

    Science.gov (United States)

    Wegner, C

    2005-04-01

    This article reviews the different pathological and immunological features of MS, acute variants of MS and acute disseminated encephalomyelitis (ADEM). T-cell-mediated inflammatory reactions are involved in all acute inflammatory diseases of the central nervous system, but the diseases discussed also exhibit distinct immunopathological features. The perivascular infiltrate of T-cells and macrophages seen in ADEM resembles the pathological pattern found in experimental autoimmune encephalomyelitis. In addition, there is evidence that humoral mechanisms play a crucial role in some acute MS lesions, Devics syndrome and Marburgs syndrome. Analysis of acute MS lesions shows many different structural and immunological features, indicating that different mechanisms may be involved in lesion formation. Distinct subtypes of acute lesions exhibit either similarities with T-cell-mediated autoimmune encephalomyelitis or signs of primary oligodendrocyte damage.

  10. [Masquerading bundle branch block].

    Science.gov (United States)

    Kukla, Piotr; Baranchuk, Adrian; Jastrzębski, Marek; Bryniarski, Leszek

    2014-01-01

    We here describe a surface 12-lead electrocardiogram (ECG) of a 72-year-old female with a prior history of breast cancer and chemotherapy-induced cardiomyopathy. An echocardiogram revealed left ventricular dysfunction, ejection fraction of 23%, with mild enlarged left ventricle. The 12-lead ECG showed atrial fibrillation with a mean heart rate of about 100 bpm, QRS duration 160 ms, QT interval 400 ms, right bundle branch block (RBBB) and left anterior fascicular block (LAFB). The combination of RBBB features in the precordial leads and LAFB features in the limb leads is known as ''masquerading bundle branch block''. In most cases of RBBB and LAFB, the QRS axis deviation is located between - 80 to -120 degrees. Rarely, when predominant left ventricular forces are present, the QRS axis deviation is near about -90 degrees, turning the pattern into an atypical form. In a situation of RBBB associated with LAFB, the S wave can be absent or very small in lead I. Such a situation is the result of not only purely LAFB but also with left ventricular hypertrophy and/or focal block due to scar (extensive anterior myocardial infarction) or fibrosis (cardiomyopathy). Sometimes, this specific ECG pattern is mistaken for LBBB. RBBB with LAFB may imitate LBBB either in the limb leads (known as 'standard masquerading' - absence of S wave in lead I), or in the precordial leads (called 'precordial masquerading' - absence of S wave in leads V₅ and V₆). Our ECG showed both these types of masquerading bundle branch block - absence of S wave in lead I and in leads V₅ and V₆.

  11. Role of Th1 and Th2 cells in autoimmune demyelinating disease

    Directory of Open Access Journals (Sweden)

    Nagelkerken L.

    1998-01-01

    Full Text Available Evidence is accumulating that Th1 cells play an important role in the development of multiple sclerosis (MS and experimental allergic encephalomyelitis (EAE, whereas Th2 cells contribute to recovery from disease. A major determinant in the development of Th1 and Th2 cells is the type of antigen-presenting cell (APC involved and its functional characteristics, e.g., the production of interleukin-12. Therefore, modulation of APC might interfere with the development of Th1 type responses and as such be beneficial for MS and EAE. The potential of cytokines, in particular interleukin-10, and glucocorticoids to exert a selective effect on APC, and as a consequence to affect the Th1-Th2 balance in EAE, is discussed

  12. Neutrophils mediate blood-spinal cord barrier disruption in demyelinating neuroinflammatory diseases.

    Science.gov (United States)

    Aubé, Benoit; Lévesque, Sébastien A; Paré, Alexandre; Chamma, Émilie; Kébir, Hania; Gorina, Roser; Lécuyer, Marc-André; Alvarez, Jorge I; De Koninck, Yves; Engelhardt, Britta; Prat, Alexandre; Côté, Daniel; Lacroix, Steve

    2014-09-01

    Disruption of the blood-brain and blood-spinal cord barriers (BBB and BSCB, respectively) and immune cell infiltration are early pathophysiological hallmarks of multiple sclerosis (MS), its animal model experimental autoimmune encephalomyelitis (EAE), and neuromyelitis optica (NMO). However, their contribution to disease initiation and development remains unclear. In this study, we induced EAE in lys-eGFP-ki mice and performed single, nonterminal intravital imaging to investigate BSCB permeability simultaneously with the kinetics of GFP(+) myeloid cell infiltration. We observed a loss in BSCB integrity within a day of disease onset, which paralleled the infiltration of GFP(+) cells into the CNS and lasted for ∼4 d. Neutrophils accounted for a significant proportion of the circulating and CNS-infiltrating myeloid cells during the preclinical phase of EAE, and their depletion delayed the onset and reduced the severity of EAE while maintaining BSCB integrity. We also show that neutrophils collected from the blood or bone marrow of EAE mice transmigrate more efficiently than do neutrophils of naive animals in a BBB cell culture model. Moreover, using intravital videomicroscopy, we demonstrate that the IL-1R type 1 governs the firm adhesion of neutrophils to the inflamed spinal cord vasculature. Finally, immunostaining of postmortem CNS material obtained from an acutely ill multiple sclerosis patient and two neuromyelitis optica patients revealed instances of infiltrated neutrophils associated with regions of BBB or BSCB leakage. Taken together, our data provide evidence that neutrophils are involved in the initial events that take place during EAE and that they are intimately linked with the status of the BBB/BSCB. Copyright © 2014 by The American Association of Immunologists, Inc.

  13. Gliopathy of Demyelinating And Non-Demyelinating Strains Of Mouse Hepatitis Virus.

    Directory of Open Access Journals (Sweden)

    Lawrence Charles Kenyon

    2015-12-01

    Full Text Available Demyelination in the central nervous system induced by neurovirulent strains of Mouse Hepatitis Virus (MHV is mediated by the viral spike glycoprotein, but it is not clear whether the mechanism of this disease pathology involves direct viral infection of oligodendrocytes. Detailed studies of glial cell tropism of MHV are presented, demonstrating that direct MHV infection of oligodendrocytes differs between demyelinating (RSA59 and non-demyelinating (RSMHV2 viral strains both in vitro and in vivo. Our results indicate that direct injury of mature oligodendrocytes is an important mechanism of virus-induced demyelination. In vivo, RSA59 infection was identified in spinal cord gray and white matter, but infected oligodendrocytes were restricted to white matter. In contrast, RSMHV2 infection was restricted to gray matter neurons and was not localized to oligodendrocytes. In vitro, RSA59 can infect both oligodendrocyte precursors and differentiated oligodendrocytes, whereas RSMHV2 can infect oligodendrocyte precursors but not differentiated oligodendrocytes. Viral spreading through axonal means to white matter and release of the demyelinating strain MHV at the nerve end is critical for oligodendrocytes infection and subsequent demyelination. Understanding the mechanisms by which known viruses effect demyelination in this animal model has important therapeutic implications in the treatment of human demyelinating disease.

  14. Chronic inflammatory demyelinative polyneuropathy

    DEFF Research Database (Denmark)

    Said, Gérard; Krarup, Christian

    2013-01-01

    Chronic inflammatory demyelinative polyneuropathy (CIDP) is an acquired polyneuropathy presumably of immunological origin. It is characterized by a progressive or a relapsing course with predominant motor deficit. The diagnosis rests on the association of non-length-dependent predominantly motor ...

  15. Transmutations in Masquerade Costumes and Performances: An ...

    African Journals Online (AJOL)

    Masquerades or spirit manifests are uniquely ritualistic. Masquerade performances in African culture are symbolizations; they represent not only the physical and continuous presence of the ancestors but also their luminal sense of justice and equity. Masquerade, derived from mask presupposes that somebody's identity is ...

  16. NFM Cross-Reactivity to MOG Does Not Expand a Critical Threshold Level of High-Affinity T Cells Necessary for Onset of Demyelinating Disease.

    Science.gov (United States)

    Blanchfield, Lori; Sabatino, Joseph J; Lawrence, Laurel; Evavold, Brian D

    2017-10-15

    Of interest to the etiology of demyelinating autoimmune disease is the potential to aberrantly activate CD4 + T cells due to cross-recognition of multiple self-epitopes such as has been suggested for myelin oligodendrocyte glycoprotein epitope 35-55 (MOG 35-55 ) and neurofilament medium protein epitope 15-35 (NFM 15-35 ). NFM 15-35  is immunogenic in C57BL/6 mice but fails to induce demyelinating disease by polyclonal T cells despite having the same TCR contact residues as MOG 35-55 , a known encephalitogenic Ag. Despite reported cross-reactivity with MOG-specific T cells, the polyclonal response to NFM 15-35 did not expand threshold numbers of MOG 38-49 tetramer-positive T cells. Furthermore, NFM lacked functional synergy with MOG to promote experimental autoimmune encephalomyelitis because NFM-deficient synonymous with knockout mice developed an identical disease course to wild-type mice after challenge with MOG 35-55 Single-cell analysis of encephalitogenic T cells using the peptide:MHC monomer-based two-dimensional micropipette adhesion frequency assay confirmed that NFM was not a critical Ag driving demyelinating disease because NFM 18-30 -specific T cells in the CNS were predominantly reactive to MOG 38-49 The absence of NFM contribution to disease allowed mapping of the amino acids required for encephalitogenicity and expansion of high-affinity, MOG-specific T cells that defined the polyclonal response. Alterations of N-terminal residues outside of the NFM 15-35 core nonamer promoted expansion of high-affinity, MOG 38-49 tetramer-positive T cells and promoted consistent experimental autoimmune encephalomyelitis induction, unlike mice challenged with NFM 15-35 Although NFM 15-35 is immunogenic and cross-reactive with MOG at the polyclonal level, it fails to expand a threshold level of encephalitogenic, high-affinity MOG-specific T cells. Copyright © 2017 by The American Association of Immunologists, Inc.

  17. Screening for SH3TC2 gene mutations in a series of demyelinating recessive Charcot-Marie-Tooth disease (CMT4).

    Science.gov (United States)

    Piscosquito, Giuseppe; Saveri, Paola; Magri, Stefania; Ciano, Claudia; Gandioli, Claudia; Morbin, Michela; Bella, Daniela D; Moroni, Isabella; Taroni, Franco; Pareyson, Davide

    2016-09-01

    Charcot-Marie-Tooth disease type 4C (CMT4C) is an autosomal recessive (AR) demyelinating neuropathy associated to SH3TC2 mutations, characterized by early onset, spine deformities, and cranial nerve involvement. We screened 43 CMT4 patients (36 index cases) with AR inheritance, demyelinating nerve conductions, and negative testing for PMP22 duplication, GJB1 and MPZ mutations, for SH3TC2 mutations. Twelve patients (11 index cases) had CMT4C as they carried homozygous or compound heterozygous mutations in SH3TC2. We found six mutations: three nonsense (p.R1109*, p.R954*, p.Q892*), one splice site (c.805+2T>C), one synonymous variant (p.K93K) predicting altered splicing, and one frameshift (p.F491Lfs*32) mutation. The splice site and the frameshift mutations are novel. Mean onset age was 7 years (range: 1-14). Neuropathy was moderate-to-severe. Scoliosis was present in 11 patients (severe in 4), and cranial nerve deficits in 9 (hearing loss in 7). Scoliosis and cranial nerve involvement are frequent features of this CMT4 subtype, and their presence should prompt the clinician to look for SH3TC2 gene mutations. In our series of undiagnosed CMT4 patients, SH3TC2 mutation frequency is 30%, confirming that CMT4C may be the most common AR-CMT type. © 2016 Peripheral Nerve Society.

  18. Central nervous system Toll-like receptor expression in response to Theiler's murine encephalomyelitis virus-induced demyelination disease in resistant and susceptible mouse strains

    Directory of Open Access Journals (Sweden)

    Turrin Nicolas P

    2008-12-01

    Full Text Available Abstract Background In immunopathological diseases, such as multiple sclerosis (MS, genetic and environmental factors that contribute to the initiation and progression of the disease are often discussed. The Theiler murine encephalomyelitis virus-induced demyelination disease (TMEV-IDD model used to study MS reflects this: genetically susceptible mice infected intra-cerebrally with TMEV develop a chronic demyelination disease. TMEV-IDD can be induced in resistant mouse strains by inducing innate immunity with lipopolysaccharide (LPS. Interestingly, Toll-like receptor 4 (TLR4 is the cognate receptor for LPS and its activation can induces up-regulation of other TLRs, such as TLR7 (the receptor for TMEV and 9, known to be involved in autoimmunity. Up-regulation of TLRs could be involved in precipitating an autoimmune susceptible state. Consequently, we looked at TLR expression in the susceptible (SJL/J and resistant (C57BL/6 strains of mice infected with TMEV. The resistant mice were induced to develop TMEV-IDD by two LPS injections following TMEV infection. Results Both strains were found to up-regulate multiple TLRs (TLR2, 7 and 9 following the TMEV infection. Expression of these TLRs and of viral mRNA was significantly greater in infected SJL/J mice. The susceptible SJL/J mice showed up-regulation of TLR3, 6 and 8, which was not seen in C57BL/6 mice. Conclusion Expression of TLRs by susceptible mice and the up-regulation of the TLRs in resistant mice could participate in priming the mice toward an autoimmune state and develop TMEV-IDD. This could have implications on therapies that target TLRs to prevent the emergence of conditions such as MS in patients at risk for the disease.

  19. Environmental factors associated with disease progression after the first demyelinating event: results from the multi-center SET study.

    Directory of Open Access Journals (Sweden)

    Dana Horakova

    Full Text Available OBJECTIVES: To investigate the associations of environmental MS risk factors with clinical and MRI measures of progression in high-risk clinically isolated syndromes (CIS after the first demyelinating event. METHODS: We analyzed 211 CIS patients (age: 28.9±7.8 years enrolled in the SET study, a multi-center study of high-risk CIS patients. Pre-treatment samples were analyzed for IgG antibodies against cytomegalovirus (anti-CMV, Epstein Barr virus (EBV early nuclear antigen-1 (EBNA-1, viral capsid antigen (VCA, early antigen-diffuse (EA-D, 25 hydroxy-vitamin D3 and cotinine levels and HLA DRB1*1501 status. The inclusion criteria required evaluation within 4 months of the initial demyelinating event, 2 or more brain MRI lesions and the presence of two or more oligoclonal bands in cerebrospinal fluid. All patients were treated with interferon-beta. Clinical and MRI assessments were obtained at baseline, 6, 12, and 24 months. RESULTS: The time to first relapse decreased and the number of relapses increased with anti-CMV IgG positivity. Smoking was associated with increased number and volume of contrast-enhancing lesions (CEL during the 2-year period. The cumulative number of CEL and T2 lesions during the 2-year period was greater for individuals in the highest quartile of anti-EBV VCA IgG antibodies. The percent loss of brain volume was increased for those in the highest quartile of with anti-EBV VCA IgG antibodies. CONCLUSIONS: Relapses in CIS patients were associated with CMV positivity whereas anti-EBV VCA positivity was associated with progression on MRI measures, including accumulation of CEL and T2 lesions and development of brain atrophy.

  20. Atypical idiopathic inflammatory demyelinating lesions

    DEFF Research Database (Denmark)

    Wallner-Blazek, Mirja; Rovira, Alex; Fillipp, Massimo

    2013-01-01

    Atypical lesions of a presumably idiopathic inflammatory demyelinating origin present quite variably and may pose diagnostic problems. The subsequent clinical course is also uncertain. We, therefore, wanted to clarify if atypical idiopathic inflammatory demyelinating lesions (AIIDLs) can be class...

  1. 22q11.2q13 duplication including SOX10 causes sex-reversal and peripheral demyelinating neuropathy, central dysmyelinating leukodystrophy, Waardenburg syndrome, and Hirschsprung disease.

    Science.gov (United States)

    Falah, Nadia; Posey, Jennifer E; Thorson, Willa; Benke, Paul; Tekin, Mustafa; Tarshish, Brocha; Lupski, James R; Harel, Tamar

    2017-04-01

    Diagnosis of genetic syndromes may be difficult when specific components of a disorder manifest at a later age. We present a follow up of a previous report [Seeherunvong et al., (2004); AJMGA 127: 149-151], of an individual with 22q duplication and sex-reversal syndrome. The subject's phenotype evolved to include peripheral and central demyelination, Waardenburg syndrome type IV, and Hirschsprung disease (PCWH; MIM 609136). DNA microarray analysis defined the duplication at 22q11.2q13, including SOX10. Sequencing of the coding region of SOX10 did not reveal any mutations. Our data suggest that SOX10 duplication can cause disorders of sex development and PCWH, supporting the hypothesis that SOX10 toxic gain of function rather than dominant negative activity underlies PCWH. © 2017 Wiley Periodicals, Inc.

  2. Aquaporin-4 Immuneglobulin G testing in 36 consecutive Jamaican patients with inflammatory central nervous system demyelinating disease

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    Sherri Sandy

    2014-08-01

    Full Text Available Epidemiological studies of neuromyelitis optica (NMO in Jamaica are lacking. Here we reviewed the clinical records of 700 patients undergoing neurological evaluation at the Kingston Public Hospital, the largest tertiary institution in Jamaica over a 4 month period. We investigated the diagnostic utility of Aquaporin-4 ImmuneglobulinG (AQP4-IgG testing in 36 consecutive patients with a diagnosis of an inflammatory demyelinating disorder (IDD of the central nervous system (CNS. Patients were classified into 3 categories: i NMO, n=10; ii multiple sclerosis (MS, n=14 and iii unclassified IDD (n=12. All sera were tested for AQP-IgG status by cell binding assay (Euroimmun. No MS cases were positive. Ninety per cent of NMO cases were positive. Four of 12 patients with unclassified IDD tested positive for AQP4-IgG. AQP4-IgG seropositivity was associated with a lower socioeconomic status, higher EDSS (P=0.04 and lower pulmonary function than the seronegative cases (P=0.007. Aquaporin-4 autoimmunity may account for a significant proportion of Jamaican CNS IDDs.

  3. Amyand's hernia masquerading inguinal abscess complicated with appendico-cutaeneous fistula in an infant with Hirschsprung's disease

    Directory of Open Access Journals (Sweden)

    Ruzaimie Noor

    2017-06-01

    Full Text Available A normal or diseased vermiform appendix located inside the inguinal hernia is called Amyand's hernia (AH. The incidence of appendicitis in Amyand's hernia is rare. The appendicitis per se is uncommon disease in infancy. We reported an extremely rare case of undiagnosed right Amyand's hernia mimicking inguinal abscess complicated with appendico-cutaneous fistula in total colonic Hirschsprung's Disease.

  4. Pachydermoperiostosis Masquerading as Acromegaly.

    Science.gov (United States)

    Karimova, Munira M; Halimova, Zamira Yu; Urmanova, Yulduz M; Korbonits, Márta; Cranston, Treena; Grossman, Ashley B

    2017-02-01

    Acromegaly usually is suspected on clinical grounds. Biochemical confirmation is required to optimize therapy, but there are other differential diagnoses. We describe a 24-year-old Uzbek man who presented with many clinical symptoms and signs of apparent acromegaly. On examination, the patient showed a rugose folding of his scalp, with the formation of tender, painful, rough skin folds in the parietal-occipital region, resembling cerebral gyri ( i.e. , cutis verticis gyrate). There was also a thickening and enlargement of the eyelids due to cartilaginous hypertrophy, dystrophic changes of the conjunctiva, and atrophy of the Meibomian glands, with the formation of multiple cysts and granulomas. He perspired excessively. There was thickening of the facial skin, with increased oiliness, increased rugosity, and seborrheic dermatitis. The skin over the hands was thick and apparently fixed to the underlying tissues. However, the patient had a low-normal insulin-like growth factor-1 level. More detailed analysis revealed a family history of relatives with similar problems, and certain features were not in keeping with this diagnosis. The disorder pachydermoperiostosis, or pulmonary hypertrophic osteoarthropathy, was suspected, and next-generation screening confirmed that the patient was homozygous for a pathogenic mutation in the SLCO2A1 gene, c.764G>A (p.Gly255Glu). The condition of pachydermoperiostosis may masquerade as acromegaly but is a genetic disorder, usually autosomal recessive, leading to elevated prostaglandin E2 levels. This is an important, albeit rare, differential diagnosis of acromegaly.

  5. Pituitary apoplexy masquerading as meningitis

    African Journals Online (AJOL)

    Pituitary apoplexy masquerading as meningitis. BJ Merwitzab*, JM Zamparinib and FJ Raalab. aCarbohydrate and Lipid Metabolism Research Unit, Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, South Africa. bCharlotte Maxeke Johannesburg Academic Hospital, Johannesburg, South Africa.

  6. Homicidal hanging masquerading as suicide

    DEFF Research Database (Denmark)

    Leth, Peter Mygind; Charles, Annie Vesterby

    1997-01-01

    Homicidal hanging is rare and presents special problems for the forensic pathologist. We report a case of homicide by hanging masquerading as suicide, in which the forensic evidence was of crucial importance. The victim was a 61 years old man, who was found in his house suspended by a rope around...

  7. Transcriptional Changes in Canine Distemper Virus-Induced Demyelinating Leukoencephalitis Favor a Biphasic Mode of Demyelination

    Science.gov (United States)

    Ulrich, Reiner; Puff, Christina; Wewetzer, Konstantin; Kalkuhl, Arno; Deschl, Ulrich; Baumgärtner, Wolfgang

    2014-01-01

    Canine distemper virus (CDV)-induced demyelinating leukoencephalitis in dogs (Canis familiaris) is suggested to represent a naturally occurring translational model for subacute sclerosing panencephalitis and multiple sclerosis in humans. The aim of this study was a hypothesis-free microarray analysis of the transcriptional changes within cerebellar specimens of five cases of acute, six cases of subacute demyelinating, and three cases of chronic demyelinating and inflammatory CDV leukoencephalitis as compared to twelve non-infected control dogs. Frozen cerebellar specimens were used for analysis of histopathological changes including demyelination, transcriptional changes employing microarrays, and presence of CDV nucleoprotein RNA and protein using microarrays, RT-qPCR and immunohistochemistry. Microarray analysis revealed 780 differentially expressed probe sets. The dominating change was an up-regulation of genes related to the innate and the humoral immune response, and less distinct the cytotoxic T-cell-mediated immune response in all subtypes of CDV leukoencephalitis as compared to controls. Multiple myelin genes including myelin basic protein and proteolipid protein displayed a selective down-regulation in subacute CDV leukoencephalitis, suggestive of an oligodendrocyte dystrophy. In contrast, a marked up-regulation of multiple immunoglobulin-like expressed sequence tags and the delta polypeptide of the CD3 antigen was observed in chronic CDV leukoencephalitis, in agreement with the hypothesis of an immune-mediated demyelination in the late inflammatory phase of the disease. Analysis of pathways intimately linked to demyelination as determined by morphometry employing correlation-based Gene Set Enrichment Analysis highlighted the pathomechanistic importance of up-regulated genes comprised by the gene ontology terms “viral replication” and “humoral immune response” as well as down-regulated genes functionally related to “metabolite and energy

  8. Transcriptional changes in canine distemper virus-induced demyelinating leukoencephalitis favor a biphasic mode of demyelination.

    Directory of Open Access Journals (Sweden)

    Reiner Ulrich

    Full Text Available Canine distemper virus (CDV-induced demyelinating leukoencephalitis in dogs (Canis familiaris is suggested to represent a naturally occurring translational model for subacute sclerosing panencephalitis and multiple sclerosis in humans. The aim of this study was a hypothesis-free microarray analysis of the transcriptional changes within cerebellar specimens of five cases of acute, six cases of subacute demyelinating, and three cases of chronic demyelinating and inflammatory CDV leukoencephalitis as compared to twelve non-infected control dogs. Frozen cerebellar specimens were used for analysis of histopathological changes including demyelination, transcriptional changes employing microarrays, and presence of CDV nucleoprotein RNA and protein using microarrays, RT-qPCR and immunohistochemistry. Microarray analysis revealed 780 differentially expressed probe sets. The dominating change was an up-regulation of genes related to the innate and the humoral immune response, and less distinct the cytotoxic T-cell-mediated immune response in all subtypes of CDV leukoencephalitis as compared to controls. Multiple myelin genes including myelin basic protein and proteolipid protein displayed a selective down-regulation in subacute CDV leukoencephalitis, suggestive of an oligodendrocyte dystrophy. In contrast, a marked up-regulation of multiple immunoglobulin-like expressed sequence tags and the delta polypeptide of the CD3 antigen was observed in chronic CDV leukoencephalitis, in agreement with the hypothesis of an immune-mediated demyelination in the late inflammatory phase of the disease. Analysis of pathways intimately linked to demyelination as determined by morphometry employing correlation-based Gene Set Enrichment Analysis highlighted the pathomechanistic importance of up-regulated genes comprised by the gene ontology terms "viral replication" and "humoral immune response" as well as down-regulated genes functionally related to "metabolite and energy

  9. Homicidal hanging masquerading as suicide

    DEFF Research Database (Denmark)

    Leth, Peter Mygind; Charles, Annie Vesterby

    1997-01-01

    his neck. The autopsy showed fresh bruises due to blunt trauma. Moreover, a voluminous subdural haematoma with brain swelling was found which indicated that the victim was unconscious at the time of the hanging. An obvious vital reaction consisting of cutaneous bleeding in the ligature mark supported......Homicidal hanging is rare and presents special problems for the forensic pathologist. We report a case of homicide by hanging masquerading as suicide, in which the forensic evidence was of crucial importance. The victim was a 61 years old man, who was found in his house suspended by a rope around...

  10. from Neuropathology and Experimental Demyelination

    Directory of Open Access Journals (Sweden)

    Graham R. Campbell

    2011-01-01

    Full Text Available Mitochondria are the most efficient producers of energy in the form of ATP. Energy demands of axons, placed at relatively great distances from the neuronal cell body, are met by mitochondria, which when functionally compromised, produce reactive oxygen species (ROS in excess. Axons are made metabolically efficient by myelination, which enables saltatory conduction. The importance of mitochondria for maintaining the structural integrity of myelinated axons is illustrated by neuroaxonal degeneration in primary mitochondrial disorders. When demyelinated, the compartmentalisation of ion channels along axons is disrupted. The redistribution of electrogenic machinery is thought to increase the energy demand of demyelinated axons. We review related studies that focus on mitochondria within unmyelinated, demyelinated and dysmyelinated axons in the central nervous system. Based on neuropathological observations we propose the increase in mitochondrial presence within demyelinated axons as an adaptive process to the increased energy need. An increased presence of mitochondria would also increase the capacity to produce deleterious agents such as ROS when functionally compromised. Given the lack of direct evidence of a beneficial or harmful effect of mitochondrial changes, the precise role of increased mitochondrial presence within axons due to demyelination needs to be further explored in experimental demyelination in-vivo and in-vitro.

  11. Uncommon Disorders Masquerading as Acute Flaccid Paralysis in Children.

    Science.gov (United States)

    Garg, Meenal; Kulkarni, Shilpa D; Patil, Varsha; Sayed, Rafat; Hegde, Anaita Udwadia

    2017-04-01

    The syndrome of acute flaccid paralysis (AFP) is a common medical emergency in children. In the era of poliomyelitis eradication, the common causes of AFP include Guillain-Barré syndrome (GBS), transverse myelitis and traumatic neuritis. However, many common diseases can uncommonly present as AFP and some uncommon diseases may also masquerade like it. Uncommon causes of AFP seen at a tertiary care pediatric hospital are discussed along with relevant points in diagnosis and management. Also, common pitfalls in diagnosis of pediatric AFP and an approach to investigations are discussed.

  12. The Prevalence of Anti-Aquaporin 4 Antibody in Patients with Idiopathic Inflammatory Demyelinating Diseases Presented to a Tertiary Hospital in Malaysia: Presentation and Prognosis

    Directory of Open Access Journals (Sweden)

    S. Abdullah

    2017-01-01

    Full Text Available Background. There have been inconsistent reports on the prevalence and pathogenicity of anti-Aquaporin 4 (AQP4 in patients presented with idiopathic inflammatory demyelinating diseases (IIDDs. Objective. To estimate the prevalence of anti-AQP4 antibody in patients with IIDDs presented to University Malaya Medical Centre in terms of patients’ clinical and radiological presentations and prognoses. Methods. Retrospective data review of IIDDs patients presented from 2005 to 2015. Patients were classified into classical multiple sclerosis (CMS, opticospinal (OS presentation, optic neuritis (ON, transverse myelitis (TM, brainstem syndrome (BS, and tumefactive MS. Anti-Aquaporin 4 antibody was tested using the Indirect Immunofluorescence Test (IIFT cell-based assay. Statistical analysis was done using the SPSS version 20. Results. Anti-AQP4 antibody was detected in 53% of patients presented with IIDDs. CMS was more common in the seronegative group, 27/47 (57.45%; p<0.001. Conversely, OS involvement was more common in the seropositive group, 26/53 (49.06%; p<0.001. Longitudinally extensive spinal cord lesions (LESCLs on MRI were also more common in the seropositive group, 29/40 (72.50%; p=0.004. Only 2/40 (5.00% had MRI evidence of patchy or multiple short-segment spinal cord lesions in the AQP4-positive group (p=0.003. The relapse rate and Expanded Disability Status Scale (EDSS were also higher in the seropositive group (5.43 versus 3.17, p=0.005; 4.07 versus 2.51, p=0.006, resp.. Typical clinical presentations that defined NMO were also seen in the seronegative patients, but in a lower frequency. Conclusion. Our cohort of patients had a higher prevalence of seropositivity of anti-AQP4 antibody as compared to those in Western countries. This was also associated with a more typical presentation of opticospinal involvement with LESCLs on MRI, a higher rate of relapse, and EDSS.

  13. Selective upregulation of scavenger receptors in and around demyelinating areas in multiple sclerosis

    NARCIS (Netherlands)

    Hendrickx, Debbie A. E.; Koning, Nathalie; Schuurman, Karianne G.; van Strien, Miriam E.; van Eden, Corbert G.; Hamann, Jörg; Huitinga, Inge

    2013-01-01

    Autoantibodies and complement opsonization have been implicated in the process of demyelination in the major human CNS demyelinating disease multiple sclerosis (MS), but scavenger receptors (SRs) may also play pathogenetic roles. We characterized SR mRNA and protein expression in postmortem brain

  14. Experimental models of demyelination and remyelination.

    Science.gov (United States)

    Torre-Fuentes, L; Moreno-Jiménez, L; Pytel, V; Matías-Guiu, J A; Gómez-Pinedo, U; Matías-Guiu, J

    2017-08-29

    Experimental animal models constitute a useful tool to deepen our knowledge of central nervous system disorders. In the case of multiple sclerosis, however, there is no such specific model able to provide an overview of the disease; multiple models covering the different pathophysiological features of the disease are therefore necessary. We reviewed the different in vitro and in vivo experimental models used in multiple sclerosis research. Concerning in vitro models, we analysed cell cultures and slice models. As for in vivo models, we examined such models of autoimmunity and inflammation as experimental allergic encephalitis in different animals and virus-induced demyelinating diseases. Furthermore, we analysed models of demyelination and remyelination, including chemical lesions caused by cuprizone, lysolecithin, and ethidium bromide; zebrafish; and transgenic models. Experimental models provide a deeper understanding of the different pathogenic mechanisms involved in multiple sclerosis. Choosing one model or another depends on the specific aims of the study. Copyright © 2017 Sociedad Española de Neurología. Publicado por Elsevier España, S.L.U. All rights reserved.

  15. The spectrum of post-vaccination inflammatory CNS demyelinating syndromes.

    Science.gov (United States)

    Karussis, Dimitrios; Petrou, Panayiota

    2014-03-01

    A wide variety of inflammatory diseases temporally associated with the administration of various vaccines, has been reported in the literature. A PubMed search from 1979 to 2013 revealed seventy one (71) documented cases. The most commonly reported vaccinations that were associated with CNS demyelinating diseases included influenza (21 cases), human papilloma virus (HPV) (9 cases), hepatitis A or B (8 cases), rabies (5 cases), measles (5 cases), rubella (5 cases), yellow fever (3 cases), anthrax (2 cases),meningococcus (2 cases) and tetanus (2 cases). The vast majority of post-vaccination CNS demyelinating syndromes, are related to influenza vaccination and this could be attributed to the high percentage of the population that received the vaccine during the HI1N1 epidemia from 2009 to 2012. Usually the symptoms of the CNS demyelinating syndrome appear few days following the immunization (mean: 14.2 days) but there are cases where the clinical presentation was delayed (more than 3 weeks or even up to 5 months post-vaccination) (approximately a third of all the reported cases). In terms of the clinical presentation and the affected CNS areas, there is a great diversity among the reported cases of post-vaccination acute demyelinating syndromes. Optic neuritis was the prominent clinical presentation in 38 cases, multifocal disseminated demyelination in 30, myelitis in 24 and encephalitis in 17. Interestingly in a rather high proportion of the patients (and especially following influenza and human papiloma virus vaccination-HPV) the dominant localizations of demyelination were the optic nerves and the myelon, presenting as optic neuritis and myelitis (with or without additional manifestations of ADEM), reminiscent to neuromyelitic optica (or, more generally, the NMO-spectrum of diseases). Seven patients suffered an NMO-like disease following HPV and we had two similar cases in our Center. One patient with post-vaccination ADEM, subsequently developed NMO. Overall, the

  16. Quantifying Demyelination in NK venom treated nerve using its electric circuit model

    Science.gov (United States)

    Das, H. K.; Das, D.; Doley, R.; Sahu, P. P.

    2016-03-01

    Reduction of myelin in peripheral nerve causes critical demyelinating diseases such as chronic inflammatory demyelinating polyneuropathy, Guillain-Barre syndrome, etc. Clinical monitoring of these diseases requires rapid and non-invasive quantification of demyelination. Here we have developed formulation of nerve conduction velocity (NCV) in terms of demyelination considering electric circuit model of a nerve having bundle of axons for its quantification from NCV measurements. This approach has been validated and demonstrated with toad nerve model treated with crude Naja kaouthia (NK) venom and also shows the effect of Phospholipase A2 and three finger neurotoxin from NK-venom on peripheral nerve. This opens future scope for non-invasive clinical measurement of demyelination.

  17. Stitch Abscess Masquerading as Recurrent Thyroid Cancer ...

    African Journals Online (AJOL)

    Recurrent thyroid cancer after remnant ablation is a known entity in follow up of differentiated thyroid cancer. It is however unusual for a stitch abscess to present as a recurrent thyroid cancer. We highlight the diagnostic dilemma of a stitch abscess masquerading as a recurrent thyroid cancer in a young female adult in the ...

  18. Differential contribution of immune effector mechanisms to cortical demyelination in multiple sclerosis.

    Science.gov (United States)

    Lagumersindez-Denis, Nielsen; Wrzos, Claudia; Mack, Matthias; Winkler, Anne; van der Meer, Franziska; Reinert, Marie C; Hollasch, Heiko; Flach, Anne; Brühl, Hilke; Cullen, Eilish; Schlumbohm, Christina; Fuchs, Eberhard; Linington, Christopher; Barrantes-Freer, Alonso; Metz, Imke; Wegner, Christiane; Liebetanz, David; Prinz, Marco; Brück, Wolfgang; Stadelmann, Christine; Nessler, Stefan

    2017-07-01

    Cortical demyelination is a widely recognized hallmark of multiple sclerosis (MS) and correlate of disease progression and cognitive decline. The pathomechanisms initiating and driving gray matter damage are only incompletely understood. Here, we determined the infiltrating leukocyte subpopulations in 26 cortical demyelinated lesions of biopsied MS patients and assessed their contribution to cortical lesion formation in a newly developed mouse model. We find that conformation-specific anti-myelin antibodies contribute to cortical demyelination even in the absence of the classical complement pathway. T cells and natural killer cells are relevant for intracortical type 2 but dispensable for subpial type 3 lesions, whereas CCR2 + monocytes are required for both. Depleting CCR2 + monocytes in marmoset monkeys with experimental autoimmune encephalomyelitis using a novel humanized CCR2 targeting antibody translates into significantly less cortical demyelination and disease severity. We conclude that biologics depleting CCR2 + monocytes might be attractive candidates for preventing cortical lesion formation and ameliorating disease progression in MS.

  19. Governance and associated social roles of masquerades among the ...

    African Journals Online (AJOL)

    The objectives of this paper are twofold. First is to verify and document governance and associated social roles played by masquerades among the Igbo of Southeast Nigeria. The second objective is to account for how globalization has impacted on such roles of masquerades over the years and to recommend the way ...

  20. 407 Costumes in Igogo Masquerade Theatre of the Ekiti People ...

    African Journals Online (AJOL)

    Regardless of the clime or culture, the physical appearance of a masquerade is a great determinant of its ... appearance that quickly suggests to its audience a simple but technically accurate combination of colours .... fashion that tilts completely on the left arm, covering the entire loins, torso and head of the masquerade, ...

  1. Visual Art Form in Motion: Traditional African masquerade as ...

    African Journals Online (AJOL)

    Masquerade is a moving art. When a masquerade performs on stage, the audience thinks mainly of the figure they see which is the visual art form. This figure is the focus of attention. But oftentimes, when people think of the works of visual arts that have to do with theatre performance, what readily comes to their mind are the ...

  2. Interferon-gamma in progression to chronic demyelination and neurological deficit following acute EAE

    DEFF Research Database (Denmark)

    Renno, T; Taupin, V; Bourbonnière, L

    1998-01-01

    The cytokine interferon-gamma (IFNgamma) is implicated in the induction of acute CNS inflammation, but it is less clear what role if any IFNgamma plays in progression to chronic demyelination and neurological deficit. To address this issue, we have expressed IFNgamma in myelinating oligodendrocyt....... IFNgamma therefore may play a role in chronic demyelination and long-term disability following the induction of demyelinating disease. Because IFNgamma may have neural as well as immune-infiltrating origins, these findings generate a new perspective on its role in the CNS....

  3. Carcinoma of Maxillary Sinus Masquerading as Odontogenic Infection.

    Science.gov (United States)

    Ramachamparambathu, Ashir Kolikkal; Vengal, Manoj; Mufeed, Abdulla; Siyo, Nizaro; Ahmed, Anis

    2016-09-01

    Malignant tumours of maxillary sinus are rare. They are usually diagnosed in the late stages when they perforate the sinus walls. The presence of large air space in the maxillary sinus facilitates asymptomatic growth of the sinus malignancy. The clinical presentation of these tumours depends on the sinus wall involved by the disease. The medial wall is usually the first to become eroded, leading to nasal obstruction, epistaxis or discharge. Rarely, symptoms of maxillary sinus carcinoma can resemble dental infection and the affected patients may visit dental clinic seeking treatment. This report presents a case of carcinoma of maxillary sinus mimicking odontogenic infection. Computed tomographic findings explained the reason for the present lesion to masquerade as an inflammatory condition. The importance of advanced imaging modalities for prompt identification of such lesions is discussed.

  4. Cerebellar white matter inflammation and demyelination in chronic relapsing experimental allergic encephalomyelitis

    DEFF Research Database (Denmark)

    Wanscher, B.; Sørensen, P. S.; Juhler, M.

    1993-01-01

    Experimental allergic encephalomyelitis, demyelination, inflammation, immunology, neuropathology......Experimental allergic encephalomyelitis, demyelination, inflammation, immunology, neuropathology...

  5. Protracted, relapsing and demyelinating experimental autoimmune encephalomyelitis in DA rats immunized with syngeneic spinal cord and incomplete Freund's adjuvant

    DEFF Research Database (Denmark)

    Lorentzen, J C; Issazadeh-Navikas, Shohreh; Storch, M

    1995-01-01

    Experimental autoimmune encephalomyelitis (EAE) is a model for multiple sclerosis (MS). However, MS is a chronic, relapsing and demyelinating disease, whereas EAE in rats is typically a brief and monophasic disorder showing little demyelination. We demonstrate here that DA rats develop severe...

  6. Demyelination precedes oligodendrocyte loss in canine distemper virus-induced encephalitis.

    Science.gov (United States)

    Schobesberger, M; Zurbriggen, A; Doherr, M G; Weissenböck, H; Vandevelde, M; Lassmann, H; Griot, C

    2002-01-01

    Canine distemper virus (CDV), a negative-stranded RNA morbillivirus, causes a persistent infection within the central nervous system resulting in a progressive, multifocal demyelinating disease. Demyelination is thought to be caused by a selective alteration of the myelin-producing oligodendrocytes. Metabolic impairment and morphological changes of the oligodendrocytes after CDV infection have previously been observed in vitro as well as in vivo. Until now it has been suggested that the oligodendrocytes completely disappear from CDV-induced demyelinating lesions. However, ultrastructural analysis in brain tissue sections and immunohistochemical examination of oligodendrocytes in dog brain cell cultures contradicted these observations. In this study oligodendrocytes from different categories of CDV-induced lesions were examined by in situ hybridization for proteolipid protein mRNA and--as a new tool employed on canine brain tissue sections--by immunohistochemistry using a monoclonal antibody against 2',3'-cyclic nucleotide 3'-phosphodiesterase, a myelin-specific enzyme. A down-regulation in the myelin gene transcription was detected already before demyelination occurred. However, a decrease in the number of oligodendrocytes was not observed until demyelination became evident. Although there was further depletion of oligodendrocytes in plaques with progressive demyelination, we demonstrated for the first time that these cells were still present in a significant amount even in chronic, completely demyelinated distemper lesions.

  7. Regional regulation of glutamate signaling during cuprizone-induced demyelination in the brain.

    Science.gov (United States)

    Azami Tameh, Abolfazl; Clarner, Tim; Beyer, Cordian; Atlasi, Mohammad Ali; Hassanzadeh, Gholamreza; Naderian, Homayoun

    2013-10-01

    Glutamate excitotoxicity is associated with a wide range of neurodegenerative disorders and also seems to be involved in the pathology of demyelinating disorders such as multiple sclerosis (MS). Cuprizone-induced toxic demyelination shows clear characteristics of MS such as demyelination and axonal damage without the involvement of the innate immune system. In this study, we have evaluated glutamate signaling during cuprizone-induced demyelination in the white and gray matter of mouse brain by studying the expression of ionotropic and metabotropic glutamate-receptors and -transporters by Affymetrix gene array analysis, followed by real-time PCR and western blot analysis. Cellular localization of glutamate transporters was investigated by fluorescence double-labeling experiments. Comparing white and gray matter areas, the expression of glutamate receptors was region-specific. Among NMDA receptor subunits, NR2A was up-regulated in the demyelinated corpus callosum (CC), whereas the metabotropic glutamate receptor mGluR2 was down-regulated in demyelinated gray matter. Glutamate-aspartate transporter (GLAST) co-localizing with GFAP(+) astrocytes was increased in both demyelinated CC and telencephalic cortex, whereas Slc1a4 transporter was up-regulated only in CC. Our data indicate that cuprizone treatment affects glutamate-receptors and -transporters differently in gray and white matter brain areas revealing particularly regulation of GLAST and Slc1a4 compared with other genes. This might have an important influence on brain-region selective sensitivity to neurotoxic compounds and the progression of demyelination as has been reported for MS and other demyelinating neurological diseases. Copyright © 2013 Elsevier GmbH. All rights reserved.

  8. Interleukin-10 overexpression promotes Fas-ligand-dependent chronic macrophage-mediated demyelinating polyneuropathy.

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    Dru S Dace

    Full Text Available BACKGROUND: Demyelinating polyneuropathy is a debilitating, poorly understood disease that can exist in acute (Guillain-Barré syndrome or chronic forms. Interleukin-10 (IL-10, although traditionally considered an anti-inflammatory cytokine, has also been implicated in promoting abnormal angiogenesis in the eye and in the pathobiology of autoimmune diseases such as lupus and encephalomyelitis. PRINCIPAL FINDINGS: Overexpression of IL-10 in a transgenic mouse model leads to macrophage-mediated demyelinating polyneuropathy. IL-10 upregulates ICAM-1 within neural tissues, promoting massive macrophage influx, inflammation-induced demyelination, and subsequent loss of neural tissue resulting in muscle weakness and paralysis. The primary insult is to perineural myelin followed by secondary axonal loss. Infiltrating macrophages within the peripheral nerves demonstrate a highly pro-inflammatory signature. Macrophages are central players in the pathophysiology, as in vivo depletion of macrophages using clodronate liposomes reverses the phenotype, including progressive nerve loss and paralysis. Macrophage-mediate demyelination is dependent on Fas-ligand (FasL-mediated Schwann cell death. SIGNIFICANCE: These findings mimic the human disease chronic idiopathic demyelinating polyneuropathy (CIDP and may also promote further understanding of the pathobiology of related conditions such as acute idiopathic demyelinating polyneuropathy (AIDP or Guillain-Barré syndrome.

  9. Auraptene induces oligodendrocyte lineage precursor cells in a cuprizone-induced animal model of demyelination.

    Science.gov (United States)

    Nakajima, Mitsunari; Shimizu, Risei; Furuta, Kohei; Sugino, Mami; Watanabe, Takashi; Aoki, Rui; Okuyama, Satoshi; Furukawa, Yoshiko

    2016-05-15

    We investigated the effects of auraptene on mouse oligodendroglial cell lineage in an animal model of demyelination induced by cuprizone. Auraptene, a citrus coumarin, was intraperitoneally administered to mice fed the demyelinating agent cuprizone. Immunohistochemical analysis of the corpus callosum and/or Western blotting analysis of brain extracts revealed that cuprizone reduced immunoreactivity for myelin-basic protein, a marker of myelin, whereas it increased immunoreactivity to platelet derived-growth factor receptor-α, a marker of oligodendrocyte precursor cells. Administration of auraptene enhanced the immunoreactivity to oligodendrocyte transcription factor 2, a marker of oligodendrocyte precursor cells and oligodendrocyte lineage precursor cells, but had no effect on immunoreactivity to myelin-basic protein or platelet-derived growth factor receptor-α. These findings suggest that auraptene promotes the production of oligodendrocyte lineage precursor cells in an animal model of demyelination and may be useful for individuals with demyelinating diseases. Copyright © 2016 Elsevier B.V. All rights reserved.

  10. Central Nervous System Demyelination and Remyelination is Independent from Systemic Cholesterol Level in Theiler's Murine Encephalomyelitis.

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    Raddatz, Barbara B; Sun, Wenhui; Brogden, Graham; Sun, Yanyong; Kammeyer, Patricia; Kalkuhl, Arno; Colbatzky, Florian; Deschl, Ulrich; Naim, Hassan Y; Baumgärtner, Wolfgang; Ulrich, Reiner

    2016-01-01

    High dietary fat and/or cholesterol intake is a risk factor for multiple diseases and has been debated for multiple sclerosis. However, cholesterol biosynthesis is a key pathway during myelination and disturbances are described in demyelinating diseases. To address the possible interaction of dyslipidemia and demyelination, cholesterol biosynthesis gene expression, composition of the body's major lipid repositories and Paigen diet-induced, systemic hypercholesterolemia were examined in Theiler's murine encephalomyelitis (TME) using histology, immunohistochemistry, serum clinical chemistry, microarrays and high-performance thin layer chromatography. TME-virus (TMEV)-infected mice showed progressive loss of motor performance and demyelinating leukomyelitis. Gene expression associated with cholesterol biosynthesis was overall down-regulated in the spinal cord of TMEV-infected animals. Spinal cord levels of galactocerebroside and sphingomyelin were reduced on day 196 post TMEV infection. Paigen diet induced serum hypercholesterolemia and hepatic lipidosis. However, high dietary fat and cholesterol intake led to no significant differences in clinical course, inflammatory response, astrocytosis, and the amount of demyelination and remyelination in the spinal cord of TMEV-infected animals. The results suggest that down-regulation of cholesterol biosynthesis is a transcriptional marker for demyelination, quantitative loss of myelin-specific lipids, but not cholesterol occurs late in chronic demyelination, and serum hypercholesterolemia exhibited no significant effect on TMEV infection. © 2015 International Society of Neuropathology.

  11. Spinal cord demyelination combined with hyperhomocysteinemia: a case report

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    Hao MM

    2014-11-01

    Full Text Available Meimei Hao, Yan Zhang, Shuangxing Hou, Yanling Chen, Ming Shi, Gang Zhao, Yanchun Deng Department of Neurology, Xijing Hospital, Fourth Military Medical University, Xi’an, People’s Republic of China Abstract: Hyperhomocysteinemia (HHcy has been recognized as an independent risk factor for atherosclerotic vascular disease. Here we report a patient who suffered from spinal cord demyelination combined with HHcy. The patient was admitted to our hospital with a diagnosis of acute myelitis. However, hormone therapy was ineffective. Further investigations revealed that he had HHcy and a homozygous mutation of the gene encoding methylenetetrahydrofolate reductase (MTHFR c.677C>T, which is a key enzyme involved in homocysteine metabolism. In view of these findings, we treated the patient with B vitamins and his symptoms gradually improved. Spinal magnetic resonance imaging performed 3 months after onset showed near recovery of the lesion. To our knowledge, similar reports are rare. Keywords: demyelination, hyperhomocysteinemia, homocysteine, methylenetetrahydrofolate reductase, methylation

  12. Intravenous immunoglobulin for chronic inflammatory demyelinating polyradiculoneuropathy

    NARCIS (Netherlands)

    Eftimov, Filip; Winer, John B.; Vermeulen, Marinus; de Haan, Rob; van Schaik, Ivo N.

    2013-01-01

    Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) causes progressive or relapsing weakness and numbness of the limbs, developing over at least two months. Uncontrolled studies suggest that intravenous immunoglobulin (IVIg) helps. This review was first published in 2002 and has since

  13. Intravenous immunoglobulin for chronic inflammatory demyelinating polyradiculoneuropathy

    NARCIS (Netherlands)

    Eftimov, Filip; Winer, John B.; Vermeulen, Marinus; de Haan, Rob; van Schaik, Ivo N.

    2009-01-01

    Background Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) causes progressive or relapsing weakness and numbness of the limbs, developing over at least two months. Uncontrolled studies suggest that intravenous immunoglobulin (IVIg) helps. Objectives To review systematically the

  14. Nogo receptor inhibition enhances functional recovery following lysolecithin-induced demyelination in mouse optic chiasm.

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    Fereshteh Pourabdolhossein

    Full Text Available Inhibitory factors have been implicated in the failure of remyelination in demyelinating diseases. Myelin associated inhibitors act through a common receptor called Nogo receptor (NgR that plays critical inhibitory roles in CNS plasticity. Here we investigated the effects of abrogating NgR inhibition in a non-immune model of focal demyelination in adult mouse optic chiasm.A focal area of demyelination was induced in adult mouse optic chiasm by microinjection of lysolecithin. To knock down NgR levels, siRNAs against NgR were intracerebroventricularly administered via a permanent cannula over 14 days, Functional changes were monitored by electrophysiological recording of latency of visual evoked potentials (VEPs. Histological analysis was carried out 3, 7 and 14 days post demyelination lesion. To assess the effect of NgR inhibition on precursor cell repopulation, BrdU was administered to the animals prior to the demyelination induction. Inhibition of NgR significantly restored VEPs responses following optic chiasm demyelination. These findings were confirmed histologically by myelin specific staining. siNgR application resulted in a smaller lesion size compared to control. NgR inhibition significantly increased the numbers of BrdU+/Olig2+ progenitor cells in the lesioned area and in the neurogenic zone of the third ventricle. These progenitor cells (Olig2+ or GFAP+ migrated away from this area as a function of time.Our results show that inhibition of NgR facilitate myelin repair in the demyelinated chiasm, with enhanced recruitment of proliferating cells to the lesion site. Thus, antagonizing NgR function could have therapeutic potential for demyelinating disorders such as Multiple Sclerosis.

  15. The topograpy of demyelination and neurodegeneration in the multiple sclerosis brain

    Science.gov (United States)

    Haider, Lukas; Hametner, Simon; Höftberger, Romana; Bagnato, Francesca; Grabner, Günther; Trattnig, Siegfried; Pfeifenbring, Sabine; Brück, Wolfgang

    2016-01-01

    Multiple sclerosis is a chronic inflammatory disease with primary demyelination and neurodegeneration in the central nervous system. In our study we analysed demyelination and neurodegeneration in a large series of multiple sclerosis brains and provide a map that displays the frequency of different brain areas to be affected by these processes. Demyelination in the cerebral cortex was related to inflammatory infiltrates in the meninges, which was pronounced in invaginations of the brain surface (sulci) and possibly promoted by low flow of the cerebrospinal fluid in these areas. Focal demyelinated lesions in the white matter occurred at sites with high venous density and additionally accumulated in watershed areas of low arterial blood supply. Two different patterns of neurodegeneration in the cortex were identified: oxidative injury of cortical neurons and retrograde neurodegeneration due to axonal injury in the white matter. While oxidative injury was related to the inflammatory process in the meninges and pronounced in actively demyelinating cortical lesions, retrograde degeneration was mainly related to demyelinated lesions and axonal loss in the white matter. Our data show that accumulation of lesions and neurodegeneration in the multiple sclerosis brain does not affect all brain regions equally and provides the pathological basis for the selection of brain areas for monitoring regional injury and atrophy development in future magnetic resonance imaging studies. PMID:26912645

  16. Extra pontine osmotic demyelination syndrome.

    Science.gov (United States)

    Zunga, Pervaiz M; Farooq, Omar; Dar, Mohd I; Dar, Ishrat H; Rashid, Samia; Rather, Abdul Q; Basu, Javid A; Ashraf, Mohammed; Bhat, Jahangeer A

    2015-01-01

    The osmotic demyelination syndrome (ODS) has been identified as a complication of the rapid correction of hyponatremia for decades. However, in recent years, a variety of other medical conditions have been associated with the development of ODS, independent of changes in serum sodium which cause a rapid changes in osmolality of the interstitial (extracellular) compartment of the brain leading to dehydration of energy-depleted cells with subsequent axonal damage that occurs in characteristic areas. Slow correction of the serum sodium concentration and additional administration of corticosteroids seems to be a major prevention step in ODS patients. In the current report we aimed to share a rare case which we observed in our hospital. A 65 year old female admitted as altered sensorium with history of vomiting, diarrhea was managed with intravenous fluids for 2 days at a peripheral health centre. Patient was referred to our centre with encephalopathy, evaluated and found to have hyponatremia and hypokalemia rest of biochemical parameters and septic profile were normal. Patient's electrolyte disturbances were managed as per guidelines but encephalopathy persisted. Supportive treatment was continued and patient was discharged after 2 wks of stay in hospital after gaining full sensorium and neurological functions.

  17. Inflammatory demyelinating neuropathy heralding accelerated chediak-higashi syndrome.

    Science.gov (United States)

    Faber, Ingrid Vasconcellos; Prota, Joana Rosa Marques; Martinez, Alberto Rolim Muro; Nucci, Anamarli; Lopes-Cendes, Iscia; Júnior, Marcondes Cavalcante França

    2017-05-01

    Chediak-Higashi syndrome (CHS) is a very rare autosomal recessive disorder (gene CHS1/LYST) characterized by partial albinism, recurrent infections, and easy bruising. Survivors develop a constellation of slowly progressive neurological manifestations. We describe clinical, laboratory, electrophysiological, and genetic findings of a patient who developed an immune-mediated demyelinating neuropathy as the main clinical feature of CHS. The patient presented with subacute flaccid paraparesis, absent reflexes, and reduced vibration sense. Protein and immunoglobulins (Igs) were elevated in the cerebrospinal fluid. Electrodiagnostic tests indicated an acquired chronic demyelinating polyneuropathy. Intravenous Ig and immunosuppressant treatment resulted in neurological improvement. The patient later developed organomegaly and pancytopenia. Bone-marrow smear revealed giant azurophilic granules pathognomonic for CHS. Two novel mutations in the LYST gene were identified through whole exome sequencing [c.7786C>T and c.9106 + 1G>T]. This case expands the clinical phenotype of CHS and highlights inflammatory demyelinating neuropathy as a manifestation of the disease. Muscle Nerve 55: 756-760, 2017. © 2016 Wiley Periodicals, Inc.

  18. Fibronectin connecting segment-1 peptide inhibits pathogenic leukocyte trafficking and inflammatory demyelination in experimental models of chronic inflammatory demyelinating polyradiculoneuropathy.

    Science.gov (United States)

    Dong, Chaoling; Greathouse, Kelsey M; Beacham, Rebecca L; Palladino, Steven P; Helton, E Scott; Ubogu, Eroboghene E

    2017-06-01

    The molecular determinants of pathogenic leukocyte migration across the blood-nerve barrier (BNB) in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) are unknown. Specific disease modifying therapies for CIDP are also lacking. Fibronectin connecting segment-1 (FNCS1), an alternatively spliced fibronectin variant expressed by microvascular endothelial cells at sites of inflammation in vitro and in situ, is a counterligand for leukocyte α 4 integrin (also known as CD49d) implicated in pathogenic leukocyte trafficking in multiple sclerosis and inflammatory bowel disease. We sought to determine the role of FNCS1 in CIDP patient leukocyte trafficking across the BNB in vitro and in severe chronic demyelinating neuritis in vivo using a representative spontaneous murine CIDP model. Peripheral blood mononuclear leukocytes from 7 untreated CIDP patients were independently infused into a cytokine-treated, flow-dependent in vitro BNB model system. Time-lapse digital video microscopy was performed to visualize and quantify leukocyte trafficking, comparing FNCS1 peptide blockade to relevant controls. Fifty 24-week old female B7-2 deficient non-obese diabetic mice with spontaneous autoimmune peripheral polyneuropathy (SAPP) were treated daily with 2mg/kg FNCS1 peptide for 5days via intraperitoneal injection with appropriate controls. Neurobehavioral measures of disease severity, motor nerve electrophysiology assessments and histopathological quantification of inflammation and morphometric assessment of demyelination were performed to determine in vivo efficacy. The biological relevance of FNCS1 and CD49d in CIDP was evaluated by immunohistochemical detection in affected patient sural nerve biopsies. 25μM FNCS1 peptide maximally inhibited CIDP leukocyte trafficking at the human BNB in vitro. FNCS1 peptide treatment resulted in significant improvements in disease severity, motor electrophysiological parameters of demyelination and histological measures of

  19. Amoebiasis masquerading as inflammatory bowel disease.

    Science.gov (United States)

    Den, Yo; Kinoshita, Junji; Deshpande, Gautam A; Hiraoka, Eiji

    2015-11-25

    A 60-year-old Japanese man presented with bloody diarrhoea. He stated that he had been diagnosed with ulcerative colitis (UC) 3 years prior, but discontinued follow-up care as treatment was ineffective. One year later, he came to our hospital with anorexia and weight loss. The abdomen was soft and flat without tenderness. Laboratory tests were unremarkable; faecal culture and Clostridium difficile toxin were negative. Findings and biopsy from a subsequent colonoscopy reconfirmed his diagnosis of UC. Neither mesalazine, which was initially prescribed, nor additional treatments improved his symptoms. Repeat colonoscopy, performed 5 months later, demonstrated similar findings in the same area. Although the pathology remained consistent with UC, multiple treatment failures suggested ongoing occult infection. Additional testing revealed positive Entamoeba histolytica antibody. 14 days of metronidazole dramatically improved his symptoms. He has remained asymptomatic after 2 years. 2015 BMJ Publishing Group Ltd.

  20. Scabies masquerading as bullous pemphigoid: scabies surrepticius

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    Cohen PR

    2017-08-01

    Full Text Available Philip R Cohen Department of Dermatology, University of California San Diego, La Jolla, CA, USA Abstract: Scabies, a parasitic infestation caused by the mite Sarcoptes scabiei, is diagnosed by observing either the mite, its ova, or its excrement. The mite tracts, known as burrows and a characteristic presentation of the pruritic condition, are typically found on the web spaces between the fingers. Other cutaneous lesions include excoriated papules, pustules, and vesicles. However, atypical clinical variants of scabies, such as bullous, crusted, hidden, incognito, nodular, and scalp forms of the parasitic infestation, mimic the morphologic features of other non-parasitic dermatoses. A 76-year-old man presented with pruritic blisters and urticarial plaques that demonstrated not only pathology changes, but direct immunofluorescence also showed findings of bullous pemphigoid. His condition improved, but did not resolve, with topical corticosteroid cream for the management of the primary autoimmune blistering disorder. When other family members subsequently developed scabies, the correct diagnosis for his condition, bullous scabies, was established by demonstrating mites, ova, and scybala on a mineral oil preparation from a skin scraping of a newly appearing burrow. Bullous scabies can masquerade not only clinically, but also both pathologically and immunologically as bullous pemphigoid. Scabies serrupticius is introduced as a unifying term to designate all of the non-classic presentations of S. scabiei mite infestation. Keywords: bullous, crusted, egg, hidden, incognito, masquerade, mimic, mite, nodular, Norwegian, pemphigoid, Sarcoptes scabiei, scabies, scalp, scybala, surrepticius

  1. Corneal sensitivity in chronic inflammatory demyelinating polyneuropathy.

    Science.gov (United States)

    Bansal, Surbhi; Myneni, Ajay A; Mu, Lina; Myers, Bennett H; Patel, Sangita P

    2014-07-01

    Neurotrophic keratitis may result from a variety of ocular or systemic diseases. Chronic inflammatory demyelinating polyneuropathy (CIDP) is an autoimmune neuropathy that affects any nerve plexus but with no known association with corneal disease. We observed 2 patients with CIDP and visually compromising neurotrophic corneal ulcers. This study was performed to determine the prevalence of neurotrophic corneas in patients with CIDP to identify a subpopulation of asymptomatic patients who are at risk for vision loss. This is an observational case series of 2 patients with CIDP with visually compromising neurotrophic corneal ulcers and a prospective clinical study comparing corneal sensitivity in 9 patients with CIDP versus 9 age- and sex-matched controls. Corneal sensitivity was tested with an esthesiometer. Statistical analyses were performed to determine patterns or significances in relation to the subject's age, gender, and duration and severity of the disease. The overall median corneal sensitivity was 5.7 for patients with CIDP and 6.0 for controls (P = 0.09). The mean corneal sensitivity was 5.6 ± 0.4 in patients with CIDP compared with 5.8 ± 0.3 in controls. No specific pattern was found with age, gender, or duration and severity of the disease among patients with CIDP. Although the case series demonstrated decreased corneal sensitivity in both patients with CIDP, the prospective study detected reduced corneal sensitivity in patients with CIDP when compared with controls, but did not reach statistical significance. Ophthalmic examinations with measurement of corneal sensitivity should be considered in the management of patients with CIDP.Clinical Trial Registration-URL: http://www.clinicaltrials.gov. Unique identifier: NCT01379833.

  2. Density-dependent predation influences the evolution and behavior of masquerading prey

    Science.gov (United States)

    Skelhorn, John; Rowland, Hannah M.; Delf, Jon; Speed, Michael P.; Ruxton, Graeme D.

    2011-01-01

    Predation is a fundamental process in the interaction between species, and exerts strong selection pressure. Hence, anti-predatory traits have been intensively studied. Although it has long been speculated that individuals of some species gain protection from predators by sometimes almost-uncanny resemblances to uninteresting objects in the local environment (such as twigs or stones), demonstration of antipredatory benefits to such “masquerade” have only very recently been demonstrated, and the fundamental workings of this defensive strategy remain unclear. Here we use laboratory experiments with avian predators and twig-mimicking caterpillars as masqueraders to investigate (i) the evolutionary dynamics of masquerade; and (ii) the behavioral adaptations associated with masquerade. We show that the benefit of masquerade declines as the local density of masqueraders relative to their models (twigs, in our system) increases. This occurs through two separate mechanisms: increasing model density both decreased predators’ motivation to search for masqueraders, and made masqueraders more difficult to detect. We further demonstrated that masquerading organisms have evolved complex microhabitat selection strategies that allow them to best exploit the density-dependent properties of masquerade. Our results strongly suggest the existence of opportunity costs associated with masquerade. Careful evaluation of such costs will be vital to the development of a fuller understanding of both the distribution of masquerade across taxa and ecosystems, and the evolution of the life history strategies of masquerading prey. PMID:21464318

  3. Autotaxin and lysophosphatidic acid1 receptor-mediated demyelination of dorsal root fibers by sciatic nerve injury and intrathecal lysophosphatidylcholine

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    Aoki Junken

    2010-11-01

    Full Text Available Abstract Background Although neuropathic pain is frequently observed in demyelinating diseases such as Guillain-Barré syndrome and multiple sclerosis, the molecular basis for the relationship between demyelination and neuropathic pain behaviors is poorly understood. Previously, we found that lysophosphatidic acid receptor (LPA1 signaling initiates sciatic nerve injury-induced neuropathic pain and demyelination. Results In the present study, we have demonstrated that sciatic nerve injury induces marked demyelination accompanied by myelin-associated glycoprotein (MAG down-regulation and damage of Schwann cell partitioning of C-fiber-containing Remak bundles in the sciatic nerve and dorsal root, but not in the spinal nerve. Demyelination, MAG down-regulation and Remak bundle damage in the dorsal root were abolished in LPA1 receptor-deficient (Lpar1-/- mice, but these alterations were not observed in sciatic nerve. However, LPA-induced demyelination in ex vivo experiments was observed in the sciatic nerve, spinal nerve and dorsal root, all which express LPA1 transcript and protein. Nerve injury-induced dorsal root demyelination was markedly attenuated in mice heterozygous for autotaxin (atx+/-, which converts lysophosphatidylcholine (LPC to LPA. Although the addition of LPC to ex vivo cultures of dorsal root fibers in the presence of recombinant ATX caused potent demyelination, it had no significant effect in the absence of ATX. On the other hand, intrathecal injection of LPC caused potent dorsal root demyelination, which was markedly attenuated or abolished in atx+/- or Lpar1-/- mice. Conclusions These results suggest that LPA, which is converted from LPC by ATX, activates LPA1 receptors and induces dorsal root demyelination following nerve injury, which causes neuropathic pain.

  4. Coexistence of two chronic neuropathies in a young child: Charcot-Marie-Tooth disease type 1A and chronic inflammatory demyelinating polyneuropathy.

    Science.gov (United States)

    Marques, Wilson; Funayama, Carolina A R; Secchin, Juliana B; Lourenço, Charles M; Gouvêa, Silmara P; Marques, Vanessa D; Bastos, Patricia G; Barreira, Amilton A

    2010-10-01

    We report an 18-month-old Charcot-Marie-Tooth type 1A (CMT1A) patient who developed a rapid-onset neuropathy, with proximal and distal weakness, and non-uniform nerve conduction studies. The neuropathy responded well to immunomodulation, confirming the coexistence of an inherited and an inflammatory neuropathy. Unexpected clinical and/or electrophysiological manifestations in CMT1A patients should alert clinicians to concomitant inflammatory neuropathy. In addition, this association raises reflections about disease mechanism in CMT1A.

  5. Monophasic demyelination reduces brain growth in children.

    Science.gov (United States)

    Aubert-Broche, Bérengère; Weier, Katrin; Longoni, Giulia; Fonov, Vladimir S; Bar-Or, Amit; Marrie, Ruth Ann; Yeh, E Ann; Narayanan, Sridar; Arnold, Douglas L; Verhey, Leonard H; Banwell, Brenda; Collins, D Louis

    2017-05-02

    To investigate how monophasic acquired demyelinating syndromes (ADS) affect age-expected brain growth over time. We analyzed 83 pediatric patients imaged serially from initial demyelinating attack: 18 with acute disseminated encephalomyelitis (ADEM) and 65 with other monophasic ADS presentations (monoADS). We further subdivided the monoADS group by the presence (n = 33; monoADSlesion) or absence (n = 32; monoADSnolesion) of T2 lesions involving the brain at onset. We used normative data to compare brain volumes and calculate age- and sex-specific z scores, and used mixed-effect models to investigate their relationship with time from demyelinating illness. Children with monophasic demyelination (ADEM, non-ADEM with brain lesions, and those without brain involvement) demonstrated reduced age-expected brain growth on serial images, driven by reduced age-expected white matter growth. Cortical gray matter volumes were not reduced at onset but demonstrated reduced age-expected growth afterwards in all groups. Brain volumes differed from age- and sex-expected values to the greatest extent in children with ADEM. All patient groups failed to recover age-expected brain growth trajectories. Brain volume, and more importantly age-expected brain growth, is negatively affected by acquired demyelination, even in the absence of chronicity, implicating factors other than active inflammation as operative in this process. © 2017 American Academy of Neurology.

  6. Cardiac sarcoid: a chameleon masquerading as hypertrophic cardiomyopathy and dilated cardiomyopathy in the same patient.

    Science.gov (United States)

    Agarwal, Anushree; Sulemanjee, Nasir Z; Cheema, Omar; Downey, Francis X; Tajik, A Jamil

    2014-05-01

    Sarcoidosis is a multisystem, granulomatous disease of unknown etiology often seen in young adults, with cardiac involvement in more than one-quarter of sarcoid patients. The clinical presentation of cardiac sarcoid depends upon the location and extent of myocardium involved. Although cardiac sarcoid may produce asymmetrical septal hypertrophy, it is most commonly considered in the differential diagnosis of dilated cardiomyopathy. The hypertrophic stage of cardiac sarcoid is rarely seen. We describe a case of cardiac sarcoid in a young patient wherein a distinctive appearance of the cardiac sarcoid spectrum from "hypertrophic" stage to thinned/scarred stage, masquerading as hypertrophic cardiomyopathy followed by dilated cardiomyopathy, is demonstrated. © 2014, Wiley Periodicals, Inc.

  7. Metastatic melanoma masquerading as a furuncle

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    Imran Aslam

    2017-10-01

    Full Text Available Melanoma metastasizes to the skin in about 10-17% of patients. Although there are reports of metastatic melanoma masquerading as panniculitis and erysipelas, it is very uncommon for it to present as an inflammatory skin lesion. When malignant melanoma cells invade the superficial dermal lymphatic vessels it can result in erythema, edema and induration of the overlying skin. This presentation can be problematic for clinicians if they do not suspect melanoma and choose not to biopsy the lesion. We report a case of an elderly man with a history of invasive melanoma who presented with a furuncle-like lesion that was found to be in-transit metastatic melanoma.

  8. Xanthogranulomatous Colitis masquerading as carcinoma of colon

    Directory of Open Access Journals (Sweden)

    Akhil Kapoor

    2015-01-01

    Full Text Available Xanthogranulomatous inflammation (XGI is an uncommon pathological diagnosis involving various organ systems, the most common being the gall bladder and kidney. It can masquerade as a malignant mass thus, requiring a clinical suspicion for accurate and timely diagnosis. A 65-year-old woman presented with acute onset of obstipation and vomiting suggesting acute obstruction. Contrast enhanced computed tomography of abdomen revealed a solid irregular mass in the ascending colon with large necrotic areas and surrounding enlarged nodes suggestive of malignancy arising from right colon. Right hemi-colectomy was performed. Histopathology of the surgical specimen showed florid inflammatory infiltrate with collection of histiocytes, lymphocytes and polymorphs. Further immunohistochemistry was conducted, and CD68 and CD45 were found to be positive and pan-cytokeratin was negative. A clinico-pathological diagnosis was thus established to be xanthogranulomatous colitis.

  9. PET imaging of demyelination and remyelination in the cuprizone mouse model for multiple sclerosis : A comparison between [C-11]CIC and [C-11]MeDAS

    NARCIS (Netherlands)

    Faria, Daniele de Paula; de Vries, Erik F. J.; Sijbesma, Jurgen W. A.; Dierckx, Rudi A. J. O.; Buchpiguel, Carlos A.; Copray, Sjef

    2014-01-01

    Multiple Sclerosis (MS) is a neurodegenerative disease characterized by demyelinated lesions. PET imaging using specific myelin radioligands might solve the lack of a specific imaging tool for diagnosing and monitoring demyelination and remyelination in MS patients. In recent years, a few tracers

  10. Acute Inflammatory Demyelinating Neuropathy : Immunoglobulin And Immune Complex Profile

    Directory of Open Access Journals (Sweden)

    Shripad A

    2003-01-01

    Full Text Available Serum immunoglobulins (IgG, IgA and IgM and immune complexes IgG (IcG were measured in 58 cases of acute inflammatory demyelinating neuropathy, popularly known as Guillian Barre′ syndrome, and in 30 healthy controls using single radial immunodiffusion assay. Immunoglobulin and immune complex levels were significantly elevated in patients as compared to controls. The increased levels of immunoglobulins and immune complexes may contribute to the pathogenesis of the disease and provide rationale for therapeutic plasmapheresis.

  11. Nail psoriasis masqueraded by secondary infection with Rhodotorula mucilaginosa.

    Science.gov (United States)

    Martini, K; Müller, H; Huemer, H P; Höpfl, R

    2013-11-01

    A 38-year-old man presented with whitish nail changes on all fingers as the sole symptom. The condition had developed within a few days and led to dystrophy of the proximal part of the nail plates. As microscopic examination of nail scrapings demonstrated budding hyphae and the patient working as a teacher reported frequent use of a wet sponge, antifungal therapy was initiated. Subsequent cultures and molecular typing identified Rhodotorula mucilaginosa (formerly R. rubra). This environmental yeast was repeatedly isolated despite of therapy with itraconazole. As no improvement was achieved and testing of the biological activity of the fungus revealed only marginal keratolytic activity, it was considered as a coloniser of a destructed nail matrix. Finally, a biopsy of the nail bed confirmed the diagnosis of nail psoriasis, which rapidly responded to treatment with acitretin and topical calcipotriol/betamethasone cream. Fungal growth in destructed nails masqueraded the underlying disease and may have triggered the psoriatic nail reaction. © 2013 Blackwell Verlag GmbH.

  12. Chronic inflammatory demyelinating polyneuropathy: a case report ...

    African Journals Online (AJOL)

    Chronic inflammatory demyelinating polyneuropathy: a case report and review of the literature. ... Vol 12, No 1 (2013) >. Log in or Register to get access to full text downloads. Username, Password, Remember me, or Register. DOWNLOAD FULL TEXT Open Access DOWNLOAD FULL TEXT Subscription or Fee Access ...

  13. Minocycline reduces remyelination by suppressing ciliary neurotrophic factor expression after cuprizone-induced demyelination.

    Science.gov (United States)

    Tanaka, Tatsuhide; Murakami, Koichi; Bando, Yoshio; Yoshida, Shigetaka

    2013-10-01

    Remyelination is disrupted in demyelinating diseases such as multiple sclerosis, but the underlying pathogenetic mechanisms are unclear. In this study, we employed the murine cuprizone model of demyelination, in which remyelination occurs after removal of the toxin from the diet, to examine the cellular and molecular changes during demyelination and remyelination. Microglia accumulated in the corpus callosum during weeks 2-4 of the cuprizone diet, and these cells remained activated 2 weeks after the change to the normal diet. To examine the role of microglia in remyelination, mice were treated with minocycline to inactivate these cells after cuprizone-induced demyelination. Minocycline treatment reduced the number of CC1-positive oligodendrocytes, as well as levels of myelin basic protein (MBP) and CNPase in the remyelination phase. The expression of CNTF mRNA in the corpus callosum increased after 4 weeks on the cuprizone diet and remained high 2 weeks after the change to the normal diet. Minocycline suppressed CNTF expression during the remyelination phase on the normal diet. Primary culture experiments showed that CNTF was produced by microglia in addition to astrocytes. In vitro, CNTF directly affected the differentiation of oligodendrocytic cells. These findings suggest that minocycline reduces remyelination by suppressing CNTF expression by microglia after cuprizone-induced demyelination. © 2013 International Society for Neurochemistry.

  14. Dermatomyositis masquerading as pulmonary embolism

    Directory of Open Access Journals (Sweden)

    Mroz RM

    2009-12-01

    Full Text Available Abstract A 61-year-old Caucasian was admitted to Department of Chest Diseases and Tuberculosis, Medical University of Bialystok, Poland for progressive muscle weakness and weight loss. Eighteen months prior to admission, the patient had been diagnosed with pulmonary embolism. At that point he was started on Enoxaparin QD. Past medical history was unremarkable. In the interim, the patient developed fever, myalgia and progressive dyspnea. Physical examination on admission revealed a rash on his upper torso and back, and the extensor surfaces of all four extremities. Laboratory values included CPK 8229, MB fraction 219, LDH 981. Chest X-ray and CT scan revealed bilateral patchy consolidations and ground-glass opacities. EMG was consistent with myositis. The patient was started on solumedrol 40 mg i.v., b.i.d., and then switched to prednisone 40 mg b.i.d. His symptoms and muscle strength improved remarkably. The patient was discharged with prednisone with an outpatient follow up.

  15. Schilder's Disease

    Science.gov (United States)

    ... See More About Research The NINDS supports and conducts an extensive research program on demyelinating disorders such as Schilder's disease. Much of this ... Publications Definition Schilder's ...

  16. In vitro analysis of the oligodendrocyte lineage in mice during demyelination and remyelination

    International Nuclear Information System (INIS)

    Armstrong, R.; Friedrich, V.L. Jr.; Holmes, K.V.; Dubois-Dalcq, M.

    1990-01-01

    A demyelinating disease induced in C57B1/6N mice by intracranial injection of a coronavirus (murine hepatitis virus strain A59) is followed by functional recovery and efficient CNS myelin repair. To study the biological properties of the cells involved in this repair process, glial cells were isolated and cultured from spinal cords of these young adult mice during demyelination and remyelination. Using three-color immunofluorescence combined with [3H]thymidine autoradiography, we have analyzed the antigenic phenotype and mitotic potential of individual glial cells. We identified oligodendrocytes with an antibody to galactocerebroside, astrocytes with an antibody to glial fibrillary acidic protein, and oligodendrocyte-type 2 astrocyte (O-2A) progenitor cells with the O4 antibody. Cultures from demyelinated tissue differed in several ways from those of age-matched controls: first, the total number of O-2A lineage cells was strikingly increased; second, the O-2A population consisted of a higher proportion of O4-positive astrocytes and cells of mixed oligodendrocyte-astrocyte phenotype; and third, all the cell types within the O-2A lineage showed enhanced proliferation. This proliferation was not further enhanced by adding PDGF, basic fibroblast growth factor (bFGF), or insulin-like growth factor I (IGF-I) to the defined medium. However, bFGF and IGF-I seemed to influence the fate of O-2A lineage cells in cultures of demyelinated tissue. Basic FGF decreased the percentage of cells expressing galactocerebroside. In contrast, IGF-I increased the relative proportion of oligodendrocytes. Thus, O-2A lineage cells from adult mice display greater phenotypic plasticity and enhanced mitotic potential in response to an episode of demyelination. These properties may be linked to the efficient remyelination achieved in this demyelinating disease

  17. Masquerade Syndrome of Multicentre Primary Central Nervous System Lymphoma

    Directory of Open Access Journals (Sweden)

    Silvana Guerriero

    2011-01-01

    Full Text Available Purpose. In Italy we say that the most unlucky things can happen to physicians when they get sick, despite the attention of colleagues. To confirm this rumor, we report the sad story of a surgeon with bilateral vitreitis and glaucoma unresponsive to traditional therapies. Methods/Design. Case report. Results. After one year of steroidal and immunosuppressive therapy, a vitrectomy, and a trabeculectomy for unresponsive bilateral vitreitis and glaucoma, MRI showed a multicentre primary central nervous system lymphoma, which was the underlying cause of the masquerade syndrome. Conclusions. All ophthalmologists and clinicians must be aware of masquerade syndromes, in order to avoid delays in diagnosis.

  18. Serum IgG levels in IV immunoglobulin treated chronic inflammatory demyelinating polyneuropathy

    NARCIS (Netherlands)

    K. Kuitwaard (Krista); P.A. van Doorn (Pieter); M. Vermeulen (Marinus); L.H. van den Berg (Leonard); E. Brusse (Esther); A.J. Kooj (Anneke); W.L. van der Pol (Ludo); I.N. van Schaik (Ivo); N.C. Notermans (Nicolette); A.P. Tio-Gillen (Anne); W. van Rijs (Wouter); T. van Gelder (Teun); B.C. Jacobs (Bart)

    2013-01-01

    textabstractObjective: To determine the variability of serum IgG in patients with chronic inflammatory demyelinating polyneuropathy (CIDP). Methods: All 25 CIDP patients had active but stable disease and were treated with individually optimised fixed dose IVIg regimens. IgG was measured by

  19. Serum IgG levels in IV immunoglobulin treated chronic inflammatory demyelinating polyneuropathy

    NARCIS (Netherlands)

    Kuitwaard, Krista; van Doorn, Pieter A.; Vermeulen, Marinus; van den Berg, Leonard H.; Brusse, Esther; van der Kooi, Anneke J.; van der Pol, W.-Ludo; van Schaik, Ivo N.; Notermans, Nicolette; Tio-Gillen, Anne P.; van Rijs, Wouter; van Gelder, Teun; Jacobs, Bart C.

    2013-01-01

    Objective To determine the variability of serum IgG in patients with chronic inflammatory demyelinating polyneuropathy (CIDP). Methods All 25 CIDP patients had active but stable disease and were treated with individually optimised fixed dose IVIg regimens. IgG was measured by turbidimetry and

  20. Bioluminescence Imaging of Olig2-Neural Stem Cells Reveals Improved Engraftment in a Demyelination Mouse Model

    NARCIS (Netherlands)

    Sher, Falak; van Dam, Go; Boddeke, Erik; Copray, Sjef

    2009-01-01

    A major issue in the potential application of neural stem cell (NSC)-based cell replacement therapy for demyelinating diseases is the question of the survival, functional behavior, and stability of implanted NSC-derived oligodendrocyte precursor cells (OPCs) over an extended period. To address this

  1. Treatments for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP): an overview of systematic reviews

    NARCIS (Netherlands)

    Oaklander, Anne Louise; Lunn, Michael Pt; Hughes, Richard Ac; van Schaik, Ivo N.; Frost, Chris; Chalk, Colin H.

    2017-01-01

    Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a chronic progressive or relapsing and remitting disease that usually causes weakness and sensory loss. The symptoms are due to autoimmune inflammation of peripheral nerves. CIPD affects about 2 to 3 per 100,000 of the population.

  2. Scabies masquerading as bullous pemphigoid: scabies surrepticius

    Science.gov (United States)

    Cohen, Philip R

    2017-01-01

    Scabies, a parasitic infestation caused by the mite Sarcoptes scabiei, is diagnosed by observing either the mite, its ova, or its excrement. The mite tracts, known as burrows and a characteristic presentation of the pruritic condition, are typically found on the web spaces between the fingers. Other cutaneous lesions include excoriated papules, pustules, and vesicles. However, atypical clinical variants of scabies, such as bullous, crusted, hidden, incognito, nodular, and scalp forms of the parasitic infestation, mimic the morphologic features of other non-parasitic dermatoses. A 76-year-old man presented with pruritic blisters and urticarial plaques that demonstrated not only pathology changes, but direct immunofluorescence also showed findings of bullous pemphigoid. His condition improved, but did not resolve, with topical corticosteroid cream for the management of the primary autoimmune blistering disorder. When other family members subsequently developed scabies, the correct diagnosis for his condition, bullous scabies, was established by demonstrating mites, ova, and scybala on a mineral oil preparation from a skin scraping of a newly appearing burrow. Bullous scabies can masquerade not only clinically, but also both pathologically and immunologically as bullous pemphigoid. Scabies serrupticius is introduced as a unifying term to designate all of the non-classic presentations of S. scabiei mite infestation. PMID:28883737

  3. Two cases of uveitis masquerade syndrome caused by bilateral intraocular large B-cell lymphoma

    Directory of Open Access Journals (Sweden)

    Jovanović Svetlana

    2013-01-01

    Full Text Available Introduction. Sometimes it is not easy to clinically recognize subtle differences between intraocular lymphoma and noninfectious uveitis. The most common lymphoma subtype involving the eye is B-cell lymphoma. Case report. We presented two patients aged 59 and 58 years with infiltration of the subretinal space with a large B-cell non-Hodgkin intraocular lymphoma. The patients originally had clinically masked syndrome in the form of intermediate uveitis. As it was a corticosteroid-resistant uveitis, we focused on the possible diagnosis of neoplastic causes of this syndrome. During hospitalization, the neurological symptoms emerged and multiple subretinal changes accompanied by yellowish white patches of retinal pigment epithelium with signs of vitritis, which made us suspect the intraocular lymphoma. Endocranial magnetic resonance imaging established tumorous infiltration in the region of the left hemisphere of the cerebellum. The histopathological finding confirmed the diagnosis of large B-cell non-Hodgkin lymphoma of risk moderate degree, immunoblast - centroblast cytological type. The other patient had clinical chronic uveitis accompanied by yellowish shaped white echographic changes of the retina and localized changes in the level of the subretina. The diagnosis of lymphoma was made by brain biopsy. Conclusion. Uveitis masquerade syndrome should be considered in all patients over 40 years with idiopathic steroid-resistant uveitis. Treatment begun on time can affect the course and improve the prognosis of uveitis masquerade syndrome (UMS and systemic disease.

  4. Dynamic masquerade with morphing three-dimensional skin in cuttlefish.

    Science.gov (United States)

    Panetta, Deanna; Buresch, Kendra; Hanlon, Roger T

    2017-03-01

    Masquerade is a defence tactic in which a prey resembles an inedible or inanimate object thus causing predators to misclassify it. Most masquerade colour patterns are static although some species adopt postures or behaviours to enhance the effect. Dynamic masquerade in which the colour pattern can be changed is rare. Here we report a two-step sensory process that enables an additional novel capability known only in cuttlefish and octopus: morphing three-dimensional physical skin texture that further enhances the optical illusions created by coloured skin patterns. Our experimental design incorporated sequential sensory processes: addition of a three-dimensional rock to the testing arena, which attracted the cuttlefish to settle next to it; then visual processing by the cuttlefish of physical textures on the rock to guide expression of the skin papillae, which can range from fully relaxed (smooth skin) to fully expressed (bumpy skin). When a uniformly white smooth rock was presented, cuttlefish moved to the rock and deployed a uniform body pattern with mostly smooth skin. When a rock with small-scale fragments of contrasting shells was presented, the cuttlefish deployed mottled body patterns with strong papillae expression. These robust and reversible responses indicate a sophisticated visual sensorimotor system for dynamic masquerade. © 2017 The Author(s).

  5. Costumes in Igogo Masquerade Theatre of the Ekiti People | Bakare ...

    African Journals Online (AJOL)

    ... heritage and sellable entertainment commodity by the younger generations of Igogo people, especially the formally educated elite. Deploying the key informant interview (KII), semiotic analysis and direct observation methods, the study highlights and discusses the socio-aesthetic attributes of Igogo masquerade theatre, ...

  6. The masquerade of death macabre in the North: strange ...

    African Journals Online (AJOL)

    The masquerade of death macabre in the North: strange revolutionary aesthetics in Nigeria. ... Against the backdrop of the current “global awakening,” this paper, through some critical works and Nigerian fictional artefacts, takes a careful examination of one particular aspect of this “harvest,” particularly the disillusionment ...

  7. Chronic inflammatory demyelinating polyradiculoneuropathy in chronic graft-versus-host disease following allogeneic hematopoietic stem cell transplantation: case report Polirradiculoneuropatia desmielinizante inflamatória crônica na doença do enxerto contra o hospedeiro após transplante de células hematopoiéticas alogênicas: relato de caso

    Directory of Open Access Journals (Sweden)

    Paulo José Lorenzoni

    2007-09-01

    Full Text Available The chronic inflammatory demyelinating polyradiculoneuropathy (CIDP is an unusual but important complication of hematopoietic stem cell transplantation (HSCT rarely reported to date. We describe a 17-year-old woman with a diagnosis of acute myeloid leukemia due to Fanconi's anemia who was submitted to allogeneic HSCT and developed CIDP as part of graft-versus-host disease. Investigation showed high cerebrospinal fluid protein; electrophysiological studies revealed sensory-motor demyelinating polyradiculoneuropathy; muscle and nerve biopsy were compatible with CIDP.A polirradiculoneuropatia desmielinizante inflamatória crônica (CIDP é uma incomum, porém, importante complicação do transplante de células hematopoiéticas (HSCT raramente relatada até a data. Nós descrevemos uma mulher de 17 anos com diagnóstico de leucemia mielóide aguda por anemia de Fanconi que foi submetida à HSCT e desenvolveu CIDP como parte da doença do enxerto contra o hospedeiro. A investigação mostrou elevação na proteína no líquor; estudo eletrofisiológico revelando polirradiculoneuropatia desmielinizante sensitivo-motora; e biópsia de músculo e nervo compatível com CIDP.

  8. A novel model of demyelination and remyelination in a GFP-transgenic zebrafish

    Directory of Open Access Journals (Sweden)

    Yangwu Fang

    2014-12-01

    Full Text Available Demyelinating diseases consist of a variety of autoimmune conditions in which the myelin sheath is damaged due to genetic and/or environmental factors. During clinical treatment, some patients undergo partial remyelination, especially during the early disease stages. However, the mechanisms that regulate demyelination remain unclear. The myelin structure, myelin formation and myelin-related gene expression are highly conserved between mammals and zebrafish. Therefore, the zebrafish is an ideal model organism to study myelination. In this study, we generated a transgenic zebrafish Tg(mbp:nfsB-egfp expressing a fusion protein composed of enhanced green fluorescent protein (EGFP and NTR from the myelin basic protein (mbp promoter. Tg(mbp:nfsB-egfp expressed NTR-EGFP reproducibly and hereditarily in oligodendrocytes along the spinal cord. Treatment of zebrafish larvae Tg(mbp:nfsB-egfp with metronidazole (Mtz resulted in the selective ablation of oligodendrocytes and led to demyelination, accompanied by behavioral changes, including decreased total movement distance, velocity, total movement time and fast movement time. After withdrawal of Mtz for a seven day recovery period, the expression of EGFP and MBP protein was observed again which indicates remyelination. Additionally, locomotor capacity was restored. Collectively, Tg(mbp:nfsB-egfp, a heritable and stable transgenic line, provides a novel, powerful tool to study the mechanisms of demyelination and remyelination.

  9. Astrocytic infection in canine distemper virus-induced demyelination.

    Science.gov (United States)

    Mutinelli, F; Vandevelde, M; Griot, C; Richard, A

    1989-01-01

    Acute canine distemper virus (CDV)-induced demyelinating lesions were examined with double-labelling immunocytochemistry simultaneously demonstrating CDV antigen and glial fibrillary acidid protein (GFAP) as marker for astrocytes. It was shown that 64% of all astrocytes within the demyelinating lesions were infected and that 95% of all infected cells counted in the lesions were astrocytes. These results suggest that the astrocyte is the main target for CDV and that astroglial infection may play an important role in the mechanism of demyelination.

  10. Acute Demyelination in a Person with Amphetamine Abuse

    Directory of Open Access Journals (Sweden)

    Serge Weis

    2011-01-01

    Full Text Available We report the case of a 31-year-old woman, admitted to the hospital for chest pain, dying a few days later from septic multiorgan failure, and showing at autopsy foci of acute demyelination in the occipital lobe. Gas chromatography/mass spectrometry analysis revealed the presence of amphetamine in the demyelinated area, which might be considered as the pathogenic agent, since other causes for demyelination could be excluded. This case represents the first report showing a demyelinating process due to a street drug.

  11. The Effect of Age on the Susceptibility and Severity of Demyelination

    Science.gov (United States)

    2015-10-01

    transgenic mouse the consequence was that these mice became sicker much quicker than a cohort of mice with normal levels of neurofascin. Therefore we...disease. In addition we challenged transgenic mice that had greatly diminished amounts of the molecule which links myelin to the axon (neurofascin) to...EAE, aging, neurofascin, transgenics , demyelinating disease, multiple sclerosis. Accomplishments- The accomplishments on this project will be

  12. Recurrent Isolated Sixth Nerve Palsy in Relapsing-Remitting Chronic Inflammatory Demyelinating Polyneuropathy.

    Science.gov (United States)

    Al-Bustani, Najwa; Weiss, Michael D

    2015-09-01

    Chronic inflammatory demyelinating polyneuropathy (CIDP) is an immune-mediated sensory and motor demyelinating polyneuropathy that typically presents as a relapsing-remitting or progressive disorder. Cranial neuropathies infrequently occur in association with other more typical symptoms of CIDP. We report a case of CIDP with recurrent isolated sixth nerve palsy. Her physical examination showed a right sixth nerve palsy and absent deep tendon reflexes as the only indicator of her disease. Magnetic resonance imaging revealed thickening without enhancement of the trigeminal and sixth cranial nerves. Nerve conduction study (NCS) revealed a sensory and motor demyelinating polyneuropathy with conduction block and temporal dispersion in multiple nerves consistent with CIDP. Cerebrospinal fluid demonstrated albuminic-cytologic dissociation. She had a remarkable response to intravenous immunoglobulin and remains asymptomatic without any additional immunomodulating therapy. Isolated cranial neuropathies can rarely occur as the sole manifestation of relapsing-remitting CIDP. The profound demyelination found on NCS in this case demonstrates that there can be a dramatic discordance between the clinical and electrodiagnostic findings in some patients with this disorder.

  13. Diagnostic algorithm for relapsing acquired demyelinating syndromes in children.

    Science.gov (United States)

    Hacohen, Yael; Mankad, Kshitij; Chong, W K; Barkhof, Frederik; Vincent, Angela; Lim, Ming; Wassmer, Evangeline; Ciccarelli, Olga; Hemingway, Cheryl

    2017-07-18

    To establish whether children with relapsing acquired demyelinating syndromes (RDS) and myelin oligodendrocyte glycoprotein antibodies (MOG-Ab) show distinctive clinical and radiologic features and to generate a diagnostic algorithm for the main RDS for clinical use. A panel reviewed the clinical characteristics, MOG-Ab and aquaporin-4 (AQP4) Ab, intrathecal oligoclonal bands, and Epstein-Barr virus serology results of 110 children with RDS. A neuroradiologist blinded to the diagnosis scored the MRI scans. Clinical, radiologic, and serologic tests results were compared. The findings showed that 56.4% of children were diagnosed with multiple sclerosis (MS), 25.4% with neuromyelitis optica spectrum disorder (NMOSD), 12.7% with multiphasic disseminated encephalomyelitis (MDEM), and 5.5% with relapsing optic neuritis (RON). Blinded analysis defined baseline MRI as typical of MS in 93.5% of children with MS. Acute disseminated encephalomyelitis presentation was seen only in the non-MS group. Of NMOSD cases, 30.7% were AQP4-Ab positive. MOG-Ab were found in 83.3% of AQP4-Ab-negative NMOSD, 100% of MDEM, and 33.3% of RON. Children with MOG-Ab were younger, were less likely to present with area postrema syndrome, and had lower disability, longer time to relapse, and more cerebellar peduncle lesions than children with AQP4-Ab NMOSD. A diagnostic algorithm applicable to any episode of CNS demyelination leads to 4 main phenotypes: MS, AQP4-Ab NMOSD, MOG-Ab-associated disease, and antibody-negative RDS. Children with MS and AQP4-Ab NMOSD showed features typical of adult cases. Because MOG-Ab-positive children showed notable and distinctive clinical and MRI features, they were grouped into a unified phenotype (MOG-Ab-associated disease), included in a new diagnostic algorithm. © 2017 American Academy of Neurology.

  14. Hyper Acute Demyelinating Encephalomyelitis of Childhood: A Rare Entity.

    Science.gov (United States)

    Kushwaha, Suman; Gupta, Ashutosh; Agarwal, Neha; Chaturvedi, Sujata; Jha, Deepak

    2017-01-01

    A young child with catastrophic neurological illness diagnosed as a rare variant of acute demyelinating encephalomyelitis (ADEM). She succumbed to her illness despite of aggressive and appropriate management. Malignant demyelinating encephalomyelitis should be considered in children who are refractory to the treatment of ADEM.

  15. Microglial recruitment, activation, and proliferation in response to primary demyelination

    DEFF Research Database (Denmark)

    Remington, Leah T; Babcock, Alicia A; Zehntner, Simone P

    2007-01-01

    microglial numbers. Microglia adopted an activated phenotype during demyelination, up-regulating major histocompatibility class I and B7.2/CD86. A subpopulation of CD45(dim-high) microglia that expressed reduced levels of CD11b emerged during demyelination. These microglia expressed CD11c and were potent...

  16. Reentrant Supraventricular Tachycardia in a Pediatric Trauma Patient Masquerading as a Cardiac Contusion

    Directory of Open Access Journals (Sweden)

    Bradbum, Christopher

    2005-01-01

    Full Text Available Establishing the etiology of tachycardia in a trauma patient is often difficult. Pediatric trauma patients present an even tougher challenge. Cardiac contusion should be suspected when other more common traumatic injuries that produce hypoxia and blood loss are excluded. The diagnosis of cardiac contusion is notoriously difficult to make largely due to the controversy over the definition of the disease, and the lack of a true gold standard confirmatory test. Atrioventricular nodal reentrant tachycardia (AVNRT is a common form of supraventricular tachycardia (SVT that can also present a diagnostic challenge to emergency physicians. While electrophysiologic studies are the gold standard for confirming the diagnosis, there are certain aspects of the history, electrocardiogram (ECG, and responses to cardiac maneuvers that strongly suggest the diagnosis. We present the case of a pediatric trauma patient that presented with new onset AVNRT masquerading as cardiac contusion.

  17. Trichilemmal Cyst of the Eyelid: Masquerading as Recurrent Chalazion

    OpenAIRE

    Meena, Manju; Mittal, Ruchi; Saha, Debarati

    2012-01-01

    A 52-years-old female presented with a history of a painless, progressive swelling in the left lower eyelid of one-year duration. The lesion was excised twice as a chalazion and recurred. Excisional biopsy of the mass was performed and histopathological findings were consistent with those of trichilemmal cyst. We report a rare case of trichilemmal cyst of the eyelid which was masquerading as chalazion for which the patient had undergone multiple surgeries. Wide excision was done and diagnosis...

  18. Dermoscopy of eccrine acrospiroma masquerading as nodular malignant melanoma.

    Science.gov (United States)

    Gatti, A; di Meo, N; Trevisan, G

    2010-12-01

    Eccrine acrospiroma, better known as eccrine poroma, is a benign adnexal neoplasm of the skin. Its clinical aspect can masquerade as some other nodular and cystic lesions. The current dermoscopy literature offers very few case studies. Moreover, these very few examples present a totally different appearance pattern compared to the one we examined. Its homogeneous blue pattern suggested the better-known nodular malignant melanoma; in fact, this dermoscopic appearance was due to the Tyndall effect.

  19. Mimicry and masquerade from the avian visual perspective

    Directory of Open Access Journals (Sweden)

    Mary Caswell STODDARD

    2012-08-01

    Full Text Available Several of the most celebrated examples of visual mimicry, like mimetic eggs laid by avian brood parasites and pala­table insects mimicking distasteful ones, involve signals directed at the eyes of birds. Despite this, studies of mimicry from the avian visual perspective have been rare, particularly with regard to defensive mimicry and masquerade. Defensive visual mimicry, which includes Batesian and Müllerian mimicry, occurs when organisms share a visual signal that functions to deter predators. Masquerade occurs when an organism mimics an inedible or uninteresting object, such as a leaf, stick, or pebble. In this paper, I present five case studies covering diverse examples of defensive mimicry and masquerade as seen by birds. The best-known cases of defensive visual mimicry typically come from insect prey, but birds themselves can exhibit defensive visual mimicry in an attempt to escape mobbing or dissuade avian predators. Using examples of defensive visual mimicry by both insects and birds, I show how quantitative models of avian color, luminance, and pattern vision can be used to enhance our understanding of mimicry in many systems and produce new hypotheses about the evolution and diversity of signals. Overall, I investigate examples of Batesian mimicry (1 and 2, Müllerian mimicry (3 and 4, and masquerade (5 as follows: 1 Polymorphic mimicry in African mocker swallowtail butterflies; 2 Cuckoos mimicking sparrowhawks; 3 Mimicry rings in Neotropical butterflies; 4 Plumage mimicry in toxic pitohuis; and 5 Dead leaf-mimicking butterflies and mantids [Current Zoology 58 (4: 630–648, 2012].

  20. Giant epidermal inclusion cyst masquerading as a soft tissue sarcoma

    Directory of Open Access Journals (Sweden)

    Hunter Phillips

    2018-02-01

    Full Text Available Epidermal inclusion cysts (EIC are common lesions formed by the invagination and cystic expansion of the epidermis or hair follicle. We present an unusual case of an epidermal inclusion cyst masquerading as a soft tissue sarcoma based on initial clinical presentation and radiographic findings. Mischaracterization of this type of lesion may lead to unnecessary diagnostic modalities or invasive, overly-aggressive surgery.

  1. Hereditary motor and sensory neuropathy with hypertrophy of the cauda equina and concomitant demyelinating white matter lesions

    International Nuclear Information System (INIS)

    Ertl-Wagner, B.B.; Staebler, A.; Reiser, M.

    2005-01-01

    Hereditary motor and sensory neuropathy (HMSN) is thought to almost exclusively affect the peripheral nervous system. We report the case of a 48-year-old patient with a longstanding history of HMSN type I who developed signs and symptoms of a cauda equina compression and of a central nervous system relapsing-remitting demyelinating white matter disease. Gross enlargement of the cauda equina fibers was detected by MR imaging of the lumbar spine. Cranial MR imaging revealed demyelinating white matter lesions. This case suggests that peripheral neuropathic mechanisms may also affect the central myelin in HMSN type I

  2. Unilateral uveitis masquerade syndrome caused by diffuse large B-cell lymphoma diagnosed using multiparametric flow cytometry of the aqueous humor.

    Science.gov (United States)

    Monsalvo, Silvia; Serrano, Cristina; Prieto, Elena; Fernández-Sanz, Guillermo; Puente, Maria-Camino; Rodriguez-Pinilla, Maria; Garcia Raso, Aranzazu; Llamas, Pilar; Cordoba, Raul

    2017-07-01

    The uveitis masquerade syndromes (UMS) are a group of ocular diseases that may mimic chronic intraocular inflammation. Many malignant entities such as non-Hodgkin's lymphomas may masquerade as uveitis. We report a case of an HIV-positive patient with masquerade syndrome presenting unilateral uveitis. 45-year-old Caucasian man with a diagnosis of diffuse large B-cell lymphoma (DLBCL). The patient was diagnosed by a biopsy of an abdominal mass which showed fragments of gastric mucosa with diffuse growth of neoplastic cells. At diagnosis, the patient suffered from unilateral blurring of vision and a sudden decrease of left-eye visual acuity. A slit-lamp examination of the left eye revealed a diagnosis of anterior uveitis. The patient exhibited no signs of posterior uveitis. An anterior-chamber paracentesis was performed and analyzed by multiparameter flow cytometry (MFC), showing cells CD45, CD19, CD20, CD22, and CD38 positives, and moderate expression of CD10 with kappa light chain restriction, showing a monoclonal B-cell population. The patient received CHOP-R with intrathecal methotrexate followed by consolidation high dose methotrexate obtaining a complete response which is ongoing. Differential diagnosis between chronic uveitis and ocular lymphoma may be challenging. We advocate anterior-chamber paracentesis in cases of refractory uveitis in patients with hematologic malignancies. © 2016 International Clinical Cytometry Society. © 2016 International Clinical Cytometry Society.

  3. Primary hyperparathyroidism may masquerade as rickets-osteomalacia in vitamin D replete children.

    Science.gov (United States)

    Ganie, Mohd Ashraf; Raizada, Nishant; Chawla, Himika; Singh, Arun Kumar; Aggarwala, Sandeep; Bal, Chandra Sekhar

    2016-10-01

    Primary hyperparathyroidism, typically a disease of the middle aged and the old, is less commonly seen in children. In children the disease has a bimodal age distribution with calcium sensing receptor mutation presenting in infancy as hypercalcemic crises and parathyroid adenoma or hyperplasia presenting later in childhood with bone disease. The childhood parathyroid adenomas are often familial with multiglandular disease and manifest with severe bone disease unlike adults. We report a series of four male patients with juvenile primary hyperparathyroidism, three of whom presented with bone disease masquerading as rickets-osteomalacia. One patient had asymptomatic hypercalcemia with short stature. Parathyroid adenoma was detected in all the four cases and all of them underwent resection of parathyroid adenomas confirmed on histopathology. Post-surgery all the cases had initial hypocalcaemia followed by normocalcemia. One case developed pancreatitis after surgery even after achieving normocalcemia. We conclude that parathyroid adenomas, although uncommon in children, are an important cause of skeletal disease that may initially be confused with hypovitaminosis D.

  4. An unusual case of osmotic demyelination syndrome without electrolyte changes in a patient with diabetes

    Directory of Open Access Journals (Sweden)

    Shrikant S Kote

    2016-01-01

    Full Text Available Central pontine myelinolysis (CPM is a demyelinating disease of the pons which is also associated with the demyelination of extrapontine areas of the central nervous system. Although the aetiology and pathogenesis are unclear, CPM is usually associated with hyponatremia or its rapid correction. Malnutrition and chronic alcoholism are also the common underlying conditions. Herein, we report a rare presentation of ODS, secondary to hyperosmolar hyperglycaemic state. We observed a 37-year-old female with diabetes type 1 and hypertension who presented with ataxia, dysarthria and pseudobulbar effect which evolved over a duration of few weeks at home with no evidence of hyponatremia or its rapid correction and no history of alcohol abuse or malnutrition.

  5. Open-ring enhancement sign in diagnosing demyelinating pseudotumor

    International Nuclear Information System (INIS)

    Fang Liting; Wang Zhiping; Wang Linyou

    2010-01-01

    Objective: To describe open-ring enhancement sign on MRI of demyelinating pseudotumor. Methods: Contrast-enhanced MRI of histologically confirmed demyelinating pseudotumors (14 patients) and astrocytomas (21) was reviewed. Results: Of the 14 cases of demyelinating pseudotumor, open-ring enhancement pattern was observed in 6; closed ring enhancement in 2; nodular enhancement in 3; patchy enhancement in 1; slight enhancement in 1; and no enhancement in 1. Of the 21 cases of astrocytoma, there was complete ring or lace-like enhancement in 13, no contrast enhancement in 6, patchy enhancement in 2, and none with open-ring enhancement pattern. Conclusion: Open-ring enhancement is a valuable sign in differential diagnosis between demyelinating pseudotumor and astrocytoma. (authors)

  6. Chronic inflammatory demyelinating polyradiculoneuropathy: from pathology to phenotype

    Science.gov (United States)

    Mathey, Emily K; Park, Susanna B; Hughes, Richard A C; Pollard, John D; Armati, Patricia J; Barnett, Michael H; Taylor, Bruce V; Dyck, P James B; Kiernan, Matthew C; Lin, Cindy S-Y

    2015-01-01

    Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an inflammatory neuropathy, classically characterised by a slowly progressive onset and symmetrical, sensorimotor involvement. However, there are many phenotypic variants, suggesting that CIDP may not be a discrete disease entity but rather a spectrum of related conditions. While the abiding theory of CIDP pathogenesis is that cell-mediated and humoral mechanisms act together in an aberrant immune response to cause damage to peripheral nerves, the relative contributions of T cell and autoantibody responses remain largely undefined. In animal models of spontaneous inflammatory neuropathy, T cell responses to defined myelin antigens are responsible. In other human inflammatory neuropathies, there is evidence of antibody responses to Schwann cell, compact myelin or nodal antigens. In this review, the roles of the cellular and humoral immune systems in the pathogenesis of CIDP will be discussed. In time, it is anticipated that delineation of clinical phenotypes and the underlying disease mechanisms might help guide diagnostic and individualised treatment strategies for CIDP. PMID:25677463

  7. Acute paretic syndrome in juvenile White Leghorn chickens resembles late stages of acute inflammatory demyelinating polyneuropathies in humans

    Directory of Open Access Journals (Sweden)

    Preisinger Rudolf

    2010-01-01

    Full Text Available Abstract Background Sudden limb paresis is a common problem in White Leghorn flocks, affecting about 1% of the chicken population before achievement of sexual maturity. Previously, a similar clinical syndrome has been reported as being caused by inflammatory demyelination of peripheral nerve fibres. Here, we investigated in detail the immunopathology of this paretic syndrome and its possible resemblance to human neuropathies. Methods Neurologically affected chickens and control animals from one single flock underwent clinical and neuropathological examination. Peripheral nervous system (PNS alterations were characterised using standard morphological techniques, including nerve fibre teasing and transmission electron microscopy. Infiltrating cells were phenotyped immunohistologically and quantified by flow cytometry. The cytokine expression pattern was assessed by quantitative real-time PCR (qRT-PCR. These investigations were accomplished by MHC genotyping and a PCR screen for Marek's disease virus (MDV. Results Spontaneous paresis of White Leghorns is caused by cell-mediated, inflammatory demyelination affecting multiple cranial and spinal nerves and nerve roots with a proximodistal tapering. Clinical manifestation coincides with the employment of humoral immune mechanisms, enrolling plasma cell recruitment, deposition of myelin-bound IgG and antibody-dependent macrophageal myelin-stripping. Disease development was significantly linked to a 539 bp microsatellite in MHC locus LEI0258. An aetiological role for MDV was excluded. Conclusions The paretic phase of avian inflammatory demyelinating polyradiculoneuritis immunobiologically resembles the late-acute disease stages of human acute inflammatory demyelinating polyneuropathy, and is characterised by a Th1-to-Th2 shift.

  8. Central canal ependymal cells proliferate extensively in response to traumatic spinal cord injury but not demyelinating lesions.

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    Steve Lacroix

    Full Text Available The adult mammalian spinal cord has limited regenerative capacity in settings such as spinal cord injury (SCI and multiple sclerosis (MS. Recent studies have revealed that ependymal cells lining the central canal possess latent neural stem cell potential, undergoing proliferation and multi-lineage differentiation following experimental SCI. To determine whether reactive ependymal cells are a realistic endogenous cell population to target in order to promote spinal cord repair, we assessed the spatiotemporal dynamics of ependymal cell proliferation for up to 35 days in three models of spinal pathologies: contusion SCI using the Infinite Horizon impactor, focal demyelination by intraspinal injection of lysophosphatidylcholine (LPC, and autoimmune-mediated multi-focal demyelination using the active experimental autoimmune encephalomyelitis (EAE model of MS. Contusion SCI at the T9-10 thoracic level stimulated a robust, long-lasting and long-distance wave of ependymal proliferation that peaked at 3 days in the lesion segment, 14 days in the rostral segment, and was still detectable at the cervical level, where it peaked at 21 days. This proliferative wave was suppressed distal to the contusion. Unlike SCI, neither chemical- nor autoimmune-mediated demyelination triggered ependymal cell proliferation at any time point, despite the occurrence of demyelination (LPC and EAE, remyelination (LPC and significant locomotor defects (EAE. Thus, traumatic SCI induces widespread and enduring activation of reactive ependymal cells, identifying them as a robust cell population to target for therapeutic manipulation after contusion; conversely, neither demyelination, remyelination nor autoimmunity appears sufficient to trigger proliferation of quiescent ependymal cells in models of MS-like demyelinating diseases.

  9. Oxidative stress and proinflammatory cytokines contribute to demyelination and axonal damage in a cerebellar culture model of neuroinflammation.

    Science.gov (United States)

    di Penta, Alessandra; Moreno, Beatriz; Reix, Stephanie; Fernandez-Diez, Begoña; Villanueva, Maite; Errea, Oihana; Escala, Nagore; Vandenbroeck, Koen; Comella, Joan X; Villoslada, Pablo

    2013-01-01

    Demyelination and axonal damage are critical processes in the pathogenesis of multiple sclerosis (MS). Oxidative stress and pro-inflammatory cytokines elicited by inflammation mediates tissue damage. To monitor the demyelination and axonal injury associated with microglia activation we employed a model using cerebellar organotypic cultures stimulated with lipopolysaccharide (LPS). Microglia activated by LPS released pro-inflammatory cytokines (IL-1β, IL-6 and TNFα), and increased the expression of inducible nitric oxide synthase (iNOS) and production of reactive oxygen species (ROS). This activation was associated with demyelination and axonal damage in cerebellar cultures. Axonal damage, as revealed by the presence of non-phosphorylated neurofilaments, mitochondrial accumulation in axonal spheroids, and axonal transection, was associated with stronger iNOS expression and concomitant increases in ROS. Moreover, we analyzed the contribution of pro-inflammatory cytokines and oxidative stress in demyelination and axonal degeneration using the iNOS inhibitor ethyl pyruvate, a free-scavenger and xanthine oxidase inhibitor allopurinol, as well as via blockage of pro-inflammatory cytokines using a Fc-TNFR1 construct. We found that blocking microglia activation with ethyl pyruvate or allopurinol significantly decreased axonal damage, and to a lesser extent, demyelination. Blocking TNFα significantly decreased demyelination but did not prevented axonal damage. Moreover, the most common therapy for MS, interferon-beta, was used as an example of an immunomodulator compound that can be tested in this model. In vitro, interferon-beta treatment decreased oxidative stress (iNOS and ROS levels) and the release of pro-inflammatory cytokines after LPS stimulation, reducing axonal damage. The model of neuroinflammation using cerebellar culture stimulated with endotoxin mimicked myelin and axonal damage mediated by the combination of oxidative stress and pro-inflammatory cytokines

  10. Trichilemmal cyst of the eyelid: masquerading as recurrent chalazion.

    Science.gov (United States)

    Meena, Manju; Mittal, Ruchi; Saha, Debarati

    2012-01-01

    A 52-years-old female presented with a history of a painless, progressive swelling in the left lower eyelid of one-year duration. The lesion was excised twice as a chalazion and recurred. Excisional biopsy of the mass was performed and histopathological findings were consistent with those of trichilemmal cyst. We report a rare case of trichilemmal cyst of the eyelid which was masquerading as chalazion for which the patient had undergone multiple surgeries. Wide excision was done and diagnosis was confirmed on histopathology. There was no recurrence seen till 2 months of postoperative period. Trichilemmal cyst, although rare, should be considered as differential diagnosis of recurrent chalazion.

  11. Trichilemmal Cyst of the Eyelid: Masquerading as Recurrent Chalazion

    Directory of Open Access Journals (Sweden)

    Manju Meena

    2012-01-01

    Full Text Available A 52-years-old female presented with a history of a painless, progressive swelling in the left lower eyelid of one-year duration. The lesion was excised twice as a chalazion and recurred. Excisional biopsy of the mass was performed and histopathological findings were consistent with those of trichilemmal cyst. We report a rare case of trichilemmal cyst of the eyelid which was masquerading as chalazion for which the patient had undergone multiple surgeries. Wide excision was done and diagnosis was confirmed on histopathology. There was no recurrence seen till 2 months of postoperative period. Trichilemmal cyst, although rare, should be considered as differential diagnosis of recurrent chalazion.

  12. Vitamin D3 attenuates oxidative stress and cognitive deficits in a model of toxic demyelination.

    Science.gov (United States)

    Tarbali, Sepideh; Khezri, Shiva

    2016-01-01

    Multiple sclerosis (MS) is a demyelinating disease. The prevalence of MS is highest where environmental supplies of vitamin D are low. Cognitive deficits have been observed in patients with MS. Oxidative damage may contribute to the formation of MS lesions. Considering the involvement of hippocampus in MS, an attempt is made in this study to investigate the effects of vitamin D3 on behavioral process and the oxidative status in the dorsal hippocampus (CA1 area) following the induction of experimental demyelination in rats. Animals were divided into six groups. animals received no surgery and treatment; saline group: animals received normal saline; sham group: animals received 150 μl sesame oil IP; vitamin D3 group: animals received 5 μg/kg vitamin D3 IP; lysophosphatidyl choline (LPC) group (toxic demyelination's model): animals received LPC by stereotaxic intra-hippocampal injection of 2 μl LPC in CA1 area; Vitamin D3- treated group: animals were treated with vitamin D3 at doses of 5 μg/kg IP for 7 and 21 days post lesion. The spatial memory, biochemical parameters including catalase (CAT) activities and lipid peroxidation levels were investigated. Animals in LPC group had more deficits in spatial memory than the control group in radial arm maze. Vitamin D3 significantly improved spatial memory compared to LPC group. Also, results indicated that vitamin D3 caused a decrease in lipid peroxidation levels and an increase in CAT activities. Current findings suggest that vitamin D3 may have a protective effect on cognitive deficits and oxidative stress in toxic demyelination's model.

  13. Probenecid-treatment reduces demyelination induced by cuprizone feeding.

    Science.gov (United States)

    Hainz, Nadine; Becker, Philipp; Rapp, Daniel; Wagenpfeil, Stefan; Wonnenberg, Bodo; Beisswenger, Christoph; Tschernig, Thomas; Meier, Carola

    2017-11-01

    Recent experiments showed that a pannexin-1 inhibitor, probenecid, reduced clinical symptoms in the murine experimental autoimmune encephalomyelitis when applied during the initial phase of neuronal inflammation. An inflammatory component is also present in a toxically induced inflammation and demyelination using cuprizone diet. Probenecid is a pannexin-1 antagonist and a probenecid therapy was investigated. Mice were fed for 10days with a cuprizone diet. In the following, the diet was continued but combined with a daily injection of a low dose of probenecid or solvent for 10days. Electron microscopy revealed demyelination in the optic nerve. The demyelination as measured by the axonal diameter was significantly reduced in the animals treated with 100mg per kg body weight probenecid. In comparison to controls, the number of leukocytes and lymphocytes in the peripheral blood was reduced in all cuprizone groups including the treatment group. In conclusion, early demyelination in the optic nerve was moderately reduced by 10days treatment with a low dose probenecid. This is a hint for the involvement of pannexin-1 modulated inflammation in cuprizone feeding induced toxic demyelination. Thus, probenecid is a candidate for the treatment of neuro-inflammation and multiple sclerosis. Copyright © 2017 Elsevier B.V. All rights reserved.

  14. Diffusion-weighted imaging in acute demyelinating myelopathy

    Energy Technology Data Exchange (ETDEWEB)

    Zecca, Chiara; Cereda, Carlo; Tschuor, Silvia; Staedler, Claudio; Nadarajah, Navarajah; Bassetti, Claudio L.; Gobbi, Claudio [Ospedale Regionale di Lugano, Servizio di Neurologia e Neuroradiologia, Neurocenter of Southern Switzerland, Lugano (Switzerland); Wetzel, Stephan [Swiss Neuro Institute (SNI), Abteilung fuer Neuroradiologie, Hirslanden Klinik Zuerich, Zuerich (Switzerland); Santini, Francesco [University of Basel Hospital, Division of Radiological Physics, Basel (Switzerland)

    2012-06-15

    Diffusion-weighted imaging (DWI) has become a reference MRI technique for the evaluation of neurological disorders. Few publications have investigated the application of DWI for inflammatory demyelinating lesions. The purpose of the study was to describe diffusion-weighted imaging characteristics of acute, spinal demyelinating lesions. Six consecutive patients (two males, four females; aged 28-64 years) with acute spinal cord demyelinating lesions were studied in a prospective case series design from June 2009 to October 2010. We performed magnetic resonance imaging studies from 2 to 14 days from symptom onset on the patients with relapsing remitting multiple sclerosis (n = 3) or clinically isolated syndrome (n = 3). Main outcome measures were diffusion-weighted imaging and apparent diffusion coefficient pattern (ADC) of acute spinal cord demyelinating lesions. All spinal lesions showed a restricted diffusion pattern (DWI+/ADC-) with a 24% median ADC signal decrease. A good correlation between clinical presentation and lesion site was observed. Acute demyelinating spinal cord lesions show a uniform restricted diffusion pattern. Clinicians and neuro-radiologists should be aware that this pattern is not necessarily confirmatory for an ischaemic aetiology. (orig.)

  15. Diffusion-weighted imaging in acute demyelinating myelopathy

    International Nuclear Information System (INIS)

    Zecca, Chiara; Cereda, Carlo; Tschuor, Silvia; Staedler, Claudio; Nadarajah, Navarajah; Bassetti, Claudio L.; Gobbi, Claudio; Wetzel, Stephan; Santini, Francesco

    2012-01-01

    Diffusion-weighted imaging (DWI) has become a reference MRI technique for the evaluation of neurological disorders. Few publications have investigated the application of DWI for inflammatory demyelinating lesions. The purpose of the study was to describe diffusion-weighted imaging characteristics of acute, spinal demyelinating lesions. Six consecutive patients (two males, four females; aged 28-64 years) with acute spinal cord demyelinating lesions were studied in a prospective case series design from June 2009 to October 2010. We performed magnetic resonance imaging studies from 2 to 14 days from symptom onset on the patients with relapsing remitting multiple sclerosis (n = 3) or clinically isolated syndrome (n = 3). Main outcome measures were diffusion-weighted imaging and apparent diffusion coefficient pattern (ADC) of acute spinal cord demyelinating lesions. All spinal lesions showed a restricted diffusion pattern (DWI+/ADC-) with a 24% median ADC signal decrease. A good correlation between clinical presentation and lesion site was observed. Acute demyelinating spinal cord lesions show a uniform restricted diffusion pattern. Clinicians and neuro-radiologists should be aware that this pattern is not necessarily confirmatory for an ischaemic aetiology. (orig.)

  16. [Correlation between demyelinating lesions and executive function decline in a sample of Mexican patients with multiple sclerosis].

    Science.gov (United States)

    Aldrete Cortez, V R; Duriez-Sotelo, E; Carrillo-Mora, P; Pérez-Zuno, J A

    2013-09-01

    Multiple Sclerosis (MS) is characterised by several neurological symptoms including cognitive impairment, which has recently been the subject of considerable study. At present, evidence pointing to a correlation between lesion characteristics and specific cognitive impairment is not conclusive. To investigate the presence of a correlation between the characteristics of demyelinating lesions and performance of basic executive functions in a sample of MS patients. We included 21 adult patients with scores of 0 to 5 on the Kurtzke scale and no exacerbations of the disease in at least 3 months prior to the evaluation date. They completed the Stroop test and the Wisconsin Card Sorting Test (WCST). The location of the lesions was determined using magnetic resonance imaging (MRI) performed by a blinded expert in neuroimaging. Demyelinating lesions were more frequently located in the frontal and occipital lobes. The Stroop test showed that as cognitive demand increased on each of the sections in the test, reaction time and number of errors increased. On the WCST, 33.33% of patients registered as having moderate cognitive impairment. No correlation could be found between demyelinating lesion characteristics (location, size, and number) and patients' scores on the tests. Explanations of the causes of cognitive impairment in MS should examine a variety of biological, psychological, and social factors instead of focusing solely on demyelinating lesions. Copyright © 2012 Sociedad Española de Neurología. Published by Elsevier Espana. All rights reserved.

  17. Deer tick masquerading as pigmented conjunctival lesion

    Directory of Open Access Journals (Sweden)

    Robin K. Kuriakose

    2017-04-01

    Conclusions and importance: Despite the low risk for Lyme disease, which is endemic to the Adirondack region where the patient was affected, doxycycline was prescribed for prophylaxis. In any case of suspected tick penetration to the ocular surface, immediate ophthalmologic consultation and prompt removal via the method mentioned above is recommended, as well as attention paid to the Infectious Diseases Society of America guidelines regarding prophylaxis.

  18. [Are there arguments for initiating an etiological treatment at the onset of the first demyelinating episode?].

    Science.gov (United States)

    Vermersch, Patrick

    2004-02-14

    The arrival of immunomodulating treatments The pathology of multiple sclerosis (MS) took benefit from progresses of magnetic resonance-based and immunological studies, leading to the introduction of immunomodulatory agents and notably interferon beta (IFNbeta). The therapeutic impact of such treatments in the relapsing-remitting forms of the disease, and our improved knowledge on the natural history of the disease, raise the possibility of treating patients after the first demyelinating episode. This strategy may be more relevant in patients presenting poor prognostic criteria. Magnetic resonance imaging (MRI) data The clinical characteristics of the first episode do not help to predict the mean or long term progression. Magnetic resonance imaging has shown that the progression of the disease is partly infra-clinical. Other than data revealing demyelination involvement and inflammation, MRI has also provided arguments suggesting early axonal distress. In a patient, the presence of high lesion load at onset on weighted T2 sequences, of black holes and gadolinium enhancing lesions s on T1 sequences, or the presence of new lesions on MRI conducted a few Months later are all predictive factors of clinical progression and suggest that treatment should be initiated early. The interest of interferon beta Two clinical trials have shown that treatment with IFNbeta treatment after a first demyelinating event significantly decreased the risk of a second episode in the short term. The aim of such treatment would be to limit the irreversible axonal lesions, notably in the form of axonal sections often associated with inflammatory lesions. An early reduction in the inflammatory response could lead to a lesser number of axons and, consequently, reduced clinical disability.

  19. Increased demyelination and axonal damage in metallothionein I+II-deficient mice during experimental autoimmune encephalomyelitis

    DEFF Research Database (Denmark)

    Penkowa, M; Espejo, C; Martínez-Cáceres, E M

    2003-01-01

    Metallothioneins I+II (MT-I+II) are antioxidant, neuroprotective factors. We previously showed that MT-I+II deficiency during experimental autoimmune encephalomyelitis (EAE) leads to increased disease incidence and clinical symptoms. Moreover, the inflammatory response of macrophages and T cells......, oxidative stress, and apoptotic cell death during EAE were increased by MT-I+II deficiency. We now show for the first time that demyelination and axonal damage are significantly increased in MT-I+II deficient mice during EAE. Furthermore, oligodendroglial regeneration, growth cone formation, and tissue...

  20. Primary Cutaneous Histoplasmosis Masquerading as Lepromatous Leprosy.

    Science.gov (United States)

    Bhattacharya, Jenna Blah; Rani, Poonam; Aggarwal, Radhika; Kaushal, Seema

    2017-01-01

    Histoplasmosis is a genus of dimorphic fungi having various varieties of which the commonest one causing infection is Histoplasma capsulatum known to cause histoplasmosis. It has a varied disease spectrum ranging from an acute infection to chronic disease especially in lungs, disseminated disease and cutaneous disorder. Histoplasma capsulatum usually causes subclinical infection and serious infections only manifest in immunocompromised patients. Frank cases of infection are seen in pulmonary histoplasmosis. The spores of these organisms are seen to be strongly associated with droppings of birds and bats. A combination of these droppings and some soil types provide for an excellent environment for the proliferation of spores. Pulmonary histoplasmosis and disseminated disease are very common in AIDS patients and are a great cause of morbidity and mortality in these patients. Primary cutaneous histoplasmosis is very rare and occurs due to penetrating injuries. Once diagnosis is made, the lesions respond very well to oral itraconazole, fluconazole or amphotericicn B. We report a rare case of Cutaneous Histoplasmosis (CHP) in a 70-year-old male with complaints of multiple nodules all over his body in a HIV seronegative and otherwise immunocompetent patient.

  1. B Cell, Th17, and Neutrophil Related Cerebrospinal Fluid Cytokine/Chemokines Are Elevated in MOG Antibody Associated Demyelination.

    Directory of Open Access Journals (Sweden)

    Kavitha Kothur

    Full Text Available Myelin oligodendrocyte glycoprotein antibody (MOG Ab associated demyelination represents a subgroup of autoimmune demyelination that is separate from multiple sclerosis and aquaporin 4 IgG-positive NMO, and can have a relapsing course. Unlike NMO and MS, there is a paucity of literature on immunopathology and CSF cytokine/chemokines in MOG Ab associated demyelination.To study the differences in immunopathogenesis based on cytokine/chemokine profile in MOG Ab-positive (POS and -negative (NEG groups.We measured 34 cytokines/chemokines using multiplex immunoassay in CSF collected from paediatric patients with serum MOG Ab POS [acute disseminated encephalomyelitis (ADEM = 8, transverse myelitis (TM = 2 n = 10] and serum MOG Ab NEG (ADEM = 5, TM = 4, n = 9 demyelination. We generated normative data using CSF from 20 non-inflammatory neurological controls.The CSF cytokine and chemokine levels were higher in both MOG Ab POS and MOG Ab NEG demyelination groups compared to controls. The CSF in MOG Ab POS patients showed predominant elevation of B cell related cytokines/chemokines (CXCL13, APRIL, BAFF and CCL19 as well as some of Th17 related cytokines (IL-6 AND G-CSF compared to MOG Ab NEG group (all p<0.01. In addition, patients with elevated CSF MOG antibodies had higher CSF CXCL13, CXCL12, CCL19, IL-17A and G-CSF than patients without CSF MOG antibodies.Our findings suggest that MOG Ab POS patients have a more pronounced CNS inflammatory response with elevation of predominant humoral associated cytokines/chemokines, as well as some Th 17 and neutrophil related cytokines/chemokines suggesting a differential inflammatory pathogenesis associated with MOG antibody seropositivity. This cytokine/chemokine profiling provides new insight into disease pathogenesis, and improves our ability to monitor inflammation and response to treatment. In addition, some of these molecules may represent potential immunomodulatory targets.

  2. Modern MRI tools for the characterization of acute demyelinating lesions: value of chemical shift and diffusion-weighted imaging

    Energy Technology Data Exchange (ETDEWEB)

    Kueker, W.; Mehnert, F.; Mader, I.; Naegele, T. [Department of Neuroradiology, University of Tuebingen Medical School, Hoppe-Seyler-Str. 3, 72076, Tuebingen (Germany); Ruff, J. [Siemens Medical Solutions, Erlangen (Germany); Gaertner, S. [Department of Neurology, University of Tuebingen Medical School, Tuebingen (Germany)

    2004-06-01

    Acute demyelinating lesions occur in various inflammatory disorders of the CNS. Apart from multiple sclerosis, most cases can be attributed to an overshooting immunological response to infectious agents called acute disseminated encephalomyelitis (ADEM). ADEM, which is mostly characterized by a monophasic course, has a multiphasic variant (MDEM). The early application of corticosteroids has been shown to be beneficial for the outcome; thus, an early diagnosis is highly desirable. Furthermore, the differential diagnosis ruling out neoplastic disorders may be difficult using conventional MRI alone. The potential diagnostic value of advanced MR techniques such as chemical shift imaging (CSI) and diffusion-weighted imaging (DWI) was investigated in a patient with MDEM, who had a new lesion in continuity with the initial disease manifestation. CSI was performed at 1.5 T with a long echo time of 135 ms for the evaluation of N-acetyl-aspartate (NAA) and choline (Cho) and with short TE of 30 ms for macromolecules (mm) and myo-Inositol (mI). DWI was performed using a single-shot isotropic EPI sequence. Whereas acute and chronic areas of demyelination were neither distinguishable on T2- nor on contrast-enhanced T1-weigted images, CSI and DWI revealed different metabolite concentrations and diffusion characteristics within the composite lesion, clearly separating acute from chronic areas of demyelination. In conclusion, the addition of CSI and DWI may add to the diagnostic power of MRI in the setting of demyelinating disorders by identifying areas of acute and chronic demyelination, even in the absence of contrast enhancement. (orig.)

  3. Modern MRI tools for the characterization of acute demyelinating lesions: value of chemical shift and diffusion-weighted imaging

    International Nuclear Information System (INIS)

    Kueker, W.; Mehnert, F.; Mader, I.; Naegele, T.; Ruff, J.; Gaertner, S.

    2004-01-01

    Acute demyelinating lesions occur in various inflammatory disorders of the CNS. Apart from multiple sclerosis, most cases can be attributed to an overshooting immunological response to infectious agents called acute disseminated encephalomyelitis (ADEM). ADEM, which is mostly characterized by a monophasic course, has a multiphasic variant (MDEM). The early application of corticosteroids has been shown to be beneficial for the outcome; thus, an early diagnosis is highly desirable. Furthermore, the differential diagnosis ruling out neoplastic disorders may be difficult using conventional MRI alone. The potential diagnostic value of advanced MR techniques such as chemical shift imaging (CSI) and diffusion-weighted imaging (DWI) was investigated in a patient with MDEM, who had a new lesion in continuity with the initial disease manifestation. CSI was performed at 1.5 T with a long echo time of 135 ms for the evaluation of N-acetyl-aspartate (NAA) and choline (Cho) and with short TE of 30 ms for macromolecules (mm) and myo-Inositol (mI). DWI was performed using a single-shot isotropic EPI sequence. Whereas acute and chronic areas of demyelination were neither distinguishable on T2- nor on contrast-enhanced T1-weigted images, CSI and DWI revealed different metabolite concentrations and diffusion characteristics within the composite lesion, clearly separating acute from chronic areas of demyelination. In conclusion, the addition of CSI and DWI may add to the diagnostic power of MRI in the setting of demyelinating disorders by identifying areas of acute and chronic demyelination, even in the absence of contrast enhancement. (orig.)

  4. A subset of fibromyalgia patients have findings suggestive of chronic inflammatory demyelinating polyneuropathy and appear to respond to IVIg.

    Science.gov (United States)

    Caro, X J; Winter, E F; Dumas, A J

    2008-02-01

    The aetiopathogenesis of the fibromyalgia syndrome (FMS) remains unknown. Recent reports, however, suggest that a subgroup of FMS subjects has an immune-mediated disease. Therefore, our primary objective was to study FMS subjects for evidence of an immune-mediated demyelinating polyneuropathy. Our secondary objective was to determine the effects of treating these FMS subjects with the immune modulator, intravenous immunoglobulin (IVIg). Fifty-eight FMS subjects, 26 rheumatic non-FMS subjects and 52 non-rheumatic non-FMS subjects were studied. Subjective measures of paraesthesias, weakness, stocking hypaesthesia, pain, fatigue and stiffness were made. Objective measures of tenderness, proximal muscle strength and electrodiagnostic (EDX) evidence of polyneuropathy and demyelination were also made. Eleven other FMS subjects underwent sural nerve biopsy. Paraesthesias, subjective weakness and stocking hypaesthesia were more common in FMS than in rheumatic non-FMS (P < or = 0.0001). Proximal muscle strength was less in FMS than in rheumatic non-FMS (P < or = 0.0001). EDX demonstrated a distal demyelinating polyneuropathy, suggestive of chronic inflammatory demyelinating polyneuropathy (CIDP), in 33% of FMS subjects. No rheumatic non-FMS subject had polyneuropathy (P = 0.005), or demyelination (P = 0.05). Fifteen FMS/CIDP subjects were subsequently treated with IVIg (400 mg/kg each day for 5 days). Pain (P = 0.01), tenderness (P = 0.001) and strength (P = 0.04) improved significantly. Fatigue and stiffness trended towards improvement. A significant subset of FMS subjects have clinical and EDX findings suggestive of CIDP. IVIg treatment shows promise in treating this subset. These observations have implications for better understanding and treating some FMS patients.

  5. Idiopathic polypoidal choroidal vasculopathy masquerading as choroidal tumors: one year follow-up of a peripheral lesion

    Directory of Open Access Journals (Sweden)

    Rony Carlos Preti

    2015-06-01

    Full Text Available ABSTRACT This case report describes peripheral idiopathic polypoidal choroidal vasculopathy (IPCV with a collection of small aneurysmal dilations that masqueraded as choroidal tumors in an elderly patient. A 68-year-old African American woman was referred to us with a suspected diagnosis of asymptomatic vascular choroidal tumor and choroidal capillary hemangioma, affecting the temporal peripheral fundus. Upon examination, optical coherence tomography (OCT revealed two large hemorrhagic pigment epithelium detachments (PED, and indocyanine green angiography (ICG confirmed the diagnosis of IPCV. One year later, there was reduction in the hemorrhagic pigment epithelium detachments and the lesion took on a different appearance, resembling a choroidal osteoma. No treatment was necessary despite the presence of multiple polyps. IPCV is a rare condition that can resemble other choroidal diseases depending on the stage of presentation. OCT is the best tool to determine the characteristics of the lesions, and indocyanine green angiography should be used to confirm the diagnosis. Not all cases require treatment.

  6. Mediastinal Choriocarcinoma Masquerading as Relapsed Hodgkin Lymphoma

    Directory of Open Access Journals (Sweden)

    Selay Lam

    2011-10-01

    Full Text Available Primary mediastinal choriocarcinoma is a rare extragonadal germ cell malignancy. We describe the first case of a patient who developed mediastinal choriocarcinoma after treatment for Hodgkin lymphoma (HL. A 25-year-old man with classic HL, nodular sclerosis subtype, underwent treatment with splenectomy followed by radiation therapy. Unfortunately, his disease relapsed with a paraspinal mass, and he was subsequently treated with MOPP (mechlorethamine, Oncovin, procarbazine, and prednisone alternating with ABVD (Adriamycin, bleomycin, vinblastine, and dacarbazine. He achieved a complete remission after 6 cycles. Ten years after treatment, the patient presented with a persistent cough, haemoptysis, right supraclavicular lymphadenopathy, and weight loss. His chest X-ray showed opacification of the lower right hemithorax with a widened mediastinum. Given unresponsiveness to several antibiotics and lack of evidence for lung volume loss, there were concerns over lung infiltration with relapsed lymphoma. Transbronchial fine needle aspiration biopsy suggested recurrence of his HL. MOPP alternating with ABVD was again given. Due to disease progression, brachytherapy as well as a cocktail of dexamethasone, cytarabine, and cisplatin were also tried. However, on a subsequent excisional lymph node biopsy, it turned out that the tumour was in fact choriocarcinoma and not relapsed HL. Unfortunately, despite aggressive therapy, the patient’s disease rapidly progressed, and he died within 2 weeks.

  7. Accumulation of Extracellular Matrix in Advanced Lesions of Canine Distemper Demyelinating Encephalitis.

    Science.gov (United States)

    Seehusen, Frauke; Al-Azreg, Seham A; Raddatz, Barbara B; Haist, Verena; Puff, Christina; Spitzbarth, Ingo; Ulrich, Reiner; Baumgärtner, Wolfgang

    2016-01-01

    In demyelinating diseases, changes in the quality and quantity of the extracellular matrix (ECM) may contribute to demyelination and failure of myelin repair and axonal sprouting, especially in chronic lesions. To characterize changes in the ECM in canine distemper demyelinating leukoencephalitis (DL), histochemical and immunohistochemical investigations of formalin-fixed paraffin-embedded cerebella using azan, picrosirius red and Gomori`s silver stain as well as antibodies directed against aggrecan, type I and IV collagen, fibronectin, laminin and phosphacan showed alterations of the ECM in CDV-infected dogs. A significantly increased amount of aggrecan was detected in early and late white matter lesions. In addition, the positive signal for collagens I and IV as well as fibronectin was significantly increased in late lesions. Conversely, the expression of phosphacan was significantly decreased in early and more pronounced in late lesions compared to controls. Furthermore, a set of genes involved in ECM was extracted from a publically available microarray data set and was analyzed for differential gene expression. Gene expression of ECM molecules, their biosynthesis pathways, and pro-fibrotic factors was mildly up-regulated whereas expression of matrix remodeling enzymes was up-regulated to a relatively higher extent. Summarized, the observed findings indicate that changes in the quality and content of ECM molecules represent important, mainly post-transcriptional features in advanced canine distemper lesions. Considering the insufficiency of morphological regeneration in chronic distemper lesions, the accumulated ECM seems to play a crucial role upon regenerative processes and may explain the relatively small regenerative potential in late stages of this disease.

  8. Identifying and quantifying recurrent novae masquerading as classical novae

    International Nuclear Information System (INIS)

    Pagnotta, Ashley; Schaefer, Bradley E.

    2014-01-01

    Recurrent novae (RNe) are cataclysmic variables with two or more nova eruptions within a century. Classical novae (CNe) are similar systems with only one such eruption. Many of the so-called CNe are actually RNe for which only one eruption has been discovered. Since RNe are candidate Type Ia supernova progenitors, it is important to know whether there are enough in our Galaxy to provide the supernova rate, and therefore to know how many RNe are masquerading as CNe. To quantify this, we collected all available information on the light curves and spectra of a Galactic, time-limited sample of 237 CNe and the 10 known RNe, as well as exhaustive discovery efficiency records. We recognize RNe as having (1) outburst amplitude smaller than 14.5 – 4.5 × log (t 3 ), (2) orbital period >0.6 days, (3) infrared colors of J – H > 0.7 mag and H – K > 0.1 mag, (4) FWHM of Hα > 2000 km s –1 , (5) high excitation lines, such as Fe X or He II near peak, (6) eruption light curves with a plateau, and (7) white dwarf mass greater than 1.2 M ☉ . Using these criteria, we identify V1721 Aql, DE Cir, CP Cru, KT Eri, V838 Her, V2672 Oph, V4160 Sgr, V4643 Sgr, V4739 Sgr, and V477 Sct as strong RN candidates. We evaluate the RN fraction among the known CNe using three methods to get 24% ± 4%, 12% ± 3%, and 35% ± 3%. With roughly a quarter of the 394 known Galactic novae actually being RNe, there should be approximately a hundred such systems masquerading as CNe.

  9. Metastatic Ovarian Tumor Masquerading as Atypical Pneumonia.

    Science.gov (United States)

    Robles Cartagena, Ivonne; Robles Cartagena, América; Delgado Flores, Glorilee; Monroig Quiles, Pedro; Cabanillas, Fernando; Báez, Luis; Luis Acabá; Santos Reyes, Luis; Vicens, Rafael

    2015-01-01

    Krukenberg tumor is a malignancy in the ovary from a primary lesion in the gastrointestinal tract and a metastatic signet ring cell adenocarcinoma to the ovary. Stomach is the most common primary site, but other organs can serve as a primary site. The lymphatic system is the most likely route for metastasis. CA 125 levels can be used for screening for early detection of ovarian metastasis as well as for monitoring the course of disease. The prognosis of Krukenberg tumor is poor and no curative treatment is currently available.

  10. Case Report: Central nervous system demyelination associated with ...

    African Journals Online (AJOL)

    Her symptoms partially resolved on cessation of the drug. Our case was unusual compared to some previous case reports, as the patient\\'s age at presentation was beyond that for idiopathic multiple sclerosis. This may strengthen the hypothesis of a causal relationship between new onset demyelination and Etanercept; ...

  11. Cavernous hemangioma in the floor of oral cavity masquerading as a ranula

    Directory of Open Access Journals (Sweden)

    Puneeta Vohra

    2015-01-01

    Full Text Available A painless, bluish, submucosal swelling on one side of the floor of the mouth usually indicates the presence of a ranula. Rarely, such a swelling may be caused by an inflammatory disease process in a salivary gland, a neoplasm in the sublingual salivary gland, hemangiomas, a lymphatic nodular swelling, amyloidosis, or embryologic cysts/dermoid cyst. We report a 35-year-old female patient with swelling in the floor of her mouth that was clinically diagnosed as a ranula due to negative diascopy, the site of swelling, and the age of patient. Because of a strong clinical suspicion of a ranula, diagnostic methods such as angiography and magnetic resonance imaging (MRI were not used in our case. A preoperative diagnosis was not truly established. We report a case of hemangioma in the floor of the mouth masquerading as a ranula. Although a rarity, vascular malformations should always be ruled out by using digital subtraction angiography and MRI studies before going for surgical intervention.

  12. Caecal tumour masquerading as an appendicular mass

    Directory of Open Access Journals (Sweden)

    Martha Nixon

    2011-12-01

    Full Text Available Appropriate management of appendix mass is based on an accurate diagnosis of the underlying pathology. This is a report of a complex patient presenting with an appendix mass, whose surgery was deferred due to severe comorbidities and who later died from severe metastatic disease. A 65-year-old lady presented with right iliac fossa pain and a mass. She was treated for an appendix mass initially and when the mass failed to resolve after four weeks, she was thoroughly investigated for the possibility of a tumour. Severe co-morbities had a significant impact on her management as definitive surgery was delayed. She represented 10 months after the initial admission with small bowel obstruction and died of metastatic caecal cancer. Management of appendix mass must entail a careful approach to investigating and treatment with emphasis on early intervention if the mass does not resolve promptly. This will avoid delayed diagnosis, treatment and a detrimental impact on prognosis.

  13. Pinworm infection masquerading as colorectal liver metastasis.

    Science.gov (United States)

    Roberts, K J; Hubscher, S; Mangat, K; Sutcliffe, R; Marudanayagam, R

    2012-09-01

    Enterobius vermicularis is responsible for a variety of diseases but rarely affects the liver. Accurate characterisation of suspected liver metastases is essential to avoid unnecessary surgery. In the presented case, following a diagnosis of rectal cancer, a solitary liver nodule was diagnosed as a liver metastasis due to typical radiological features and subsequently resected. At pathological assessment, however, a necrotic nodule containing E. vermicularis was identified. Solitary necrotic nodules of the liver are usually benign but misdiagnosed frequently as malignant due to radiological features. It is standard practice to diagnose colorectal liver metastases solely on radiological evidence. Without obtaining tissue prior to liver resection, misdiagnosis of solitary necrotic nodules of the liver will continue to occur.

  14. Knee tuberculosis masquerading as pigmented villonodular synovitis

    Directory of Open Access Journals (Sweden)

    Sanjay Meena

    2014-01-01

    Full Text Available Tuberculosis (TB, once a disease confined to undeveloped or developing nations is currently in resurgence, which is attributable to pandemic human immunodeficiency virus (HIV infection and immigration from endemic areas. Tuberculous arthritis is difficult to diagnose early because of its atypical insidious clinical manifestations and nonspecific imaging findings. TB is also known as the ′great mimicker′. Specifically, monoarticular tuberculosis of the knee may mimic pigmented villonodular synovitis (PVNS. The present report describes a young patient with tuberculous arthritis of knee joint. Accurate diagnosis and appropriate management was delayed due to magnetic resonance imaging (MRI findings, such as, hemosiderin deposits and a nodular mass around the knee joint, suggestive of a diffuse type of PVNS. Our findings suggest that the first step in the diagnosis of tuberculous knee arthritis is to have a high index of suspicion.

  15. Ophthalmic masquerades of the atherosclerotic carotids

    Directory of Open Access Journals (Sweden)

    Anupriya Arthur

    2014-01-01

    Full Text Available Patients with carotid atherosclerosis can present with ophthalmic symptoms. These symptoms and signs can be due to retinal emboli, hypoperfusion of the retina and choroid, opening up of collateral channels, or chronic hypoperfusion of the globe (ocular ischemic syndrome. These pathological mechanisms can produce many interesting signs and a careful history can bring out important past symptoms pointing toward the carotid as the source of the patient′s presenting symptom. Such patients are at high risk for an ischemic stroke, especially in the subsequent few days following their first acute symptom. It is important for clinicians to be familiar with these ophthalmic symptoms and signs caused by carotid atherosclerosis for making an early diagnosis and to take appropriate measures to prevent a stroke. This review elaborates the clinical features, importance, and implications of various ophthalmic symptoms and signs resulting from atherosclerotic carotid artery disease.

  16. Thyrotoxicosis and Choledocholithiasis Masquerading as Thyroid Storm

    Directory of Open Access Journals (Sweden)

    Christian L. Horn

    2017-01-01

    Full Text Available A 26-year-old female, thirteen months postpartum, presented to the emergency department for four weeks of epigastric abdominal pain, pruritus, new onset jaundice, and 11.3 kgs (25 lbs unintentional weight loss. On examination, she was afebrile, tachycardic, alert, and oriented and had jaundice with scleral icterus. Labs were significant for undetectable TSH, FT4 that was too high to measure, and elevated total bilirubin, direct bilirubin, alkaline phosphatase, and transaminases. Abdominal ultrasound revealed cholelithiasis without biliary ductal dilation. Treatment for presumed thyroid storm was initiated. Further work-up with magnetic resonance cholangiopancreatography (MRCP revealed an obstructing cholelith within the distal common bile duct. With the presence of choledocholithiasis explaining the jaundice and abdominal pain, plus the absence of CNS alterations, the diagnosis of thyroid storm was revised to thyrotoxicosis complicated by choledocholithiasis. Endoscopic retrograde cholangiopancreatogram (ERCP with sphincterotomy was performed to alleviate the biliary obstruction, with prompt symptomatic improvement. Thyroid storm is a rare manifestation of hyperthyroidism with a high rate of morbidity and mortality. The diagnosis of thyroid storm is based on clinical examination, and abnormal thyroid function tests do not correlate with disease severity. Knowledge of the many manifestations of thyroid storm will facilitate a quick and accurate diagnosis and treatment.

  17. Rare adipose disorders (RADs) masquerading as obesity

    Science.gov (United States)

    Herbst, Karen L

    2012-01-01

    Rare adipose disorders (RADs) including multiple symmetric lipomatosis (MSL), lipedema and Dercum's disease (DD) may be misdiagnosed as obesity. Lifestyle changes, such as reduced caloric intake and increased physical activity are standard care for obesity. Although lifestyle changes and bariatric surgery work effectively for the obesity component of RADs, these treatments do not routinely reduce the abnormal subcutaneous adipose tissue (SAT) of RADs. RAD SAT likely results from the growth of a brown stem cell population with secondary lymphatic dysfunction in MSL, or by primary vascular and lymphatic dysfunction in lipedema and DD. People with RADs do not lose SAT from caloric limitation and increased energy expenditure alone. In order to improve recognition of RADs apart from obesity, the diagnostic criteria, histology and pathophysiology of RADs are presented and contrasted to familial partial lipodystrophies, acquired partial lipodystrophies and obesity with which they may be confused. Treatment recommendations focus on evidence-based data and include lymphatic decongestive therapy, medications and supplements that support loss of RAD SAT. Associated RAD conditions including depression, anxiety and pain will improve as healthcare providers learn to identify and adopt alternative treatment regimens for the abnormal SAT component of RADs. Effective dietary and exercise regimens are needed in RAD populations to improve quality of life and construct advanced treatment regimens for future generations. PMID:22301856

  18. Acutely damaged axons are remyelinated in multiple sclerosis and experimental models of demyelination.

    Science.gov (United States)

    Schultz, Verena; van der Meer, Franziska; Wrzos, Claudia; Scheidt, Uta; Bahn, Erik; Stadelmann, Christine; Brück, Wolfgang; Junker, Andreas

    2017-08-01

    Remyelination is in the center of new therapies for the treatment of multiple sclerosis to resolve and improve disease symptoms and protect axons from further damage. Although remyelination is considered beneficial in the long term, it is not known, whether this is also the case early in lesion formation. Additionally, the precise timing of acute axonal damage and remyelination has not been assessed so far. To shed light onto the interrelation between axons and the myelin sheath during de- and remyelination, we employed cuprizone- and focal lysolecithin-induced demyelination and performed time course experiments assessing the evolution of early and late stage remyelination and axonal damage. We observed damaged axons with signs of remyelination after cuprizone diet cessation and lysolecithin injection. Similar observations were made in early multiple sclerosis lesions. To assess the correlation of remyelination and axonal damage in multiple sclerosis lesions, we took advantage of a cohort of patients with early and late stage remyelinated lesions and assessed the number of APP- and SMI32- positive damaged axons and the density of SMI31-positive and silver impregnated preserved axons. Early de- and remyelinating lesions did not differ with respect to axonal density and axonal damage, but we observed a lower axonal density in late stage demyelinated multiple sclerosis lesions than in remyelinated multiple sclerosis lesions. Our findings suggest that remyelination may not only be protective over a long period of time, but may play an important role in the immediate axonal recuperation after a demyelinating insult. © 2017 The Authors GLIA Published by Wiley Periodicals, Inc.

  19. Novel insight into Chronic Inflammatory Demyelinating Polineuropathy in APECED syndrome: molecular mechanisms and clinical implications in children.

    Science.gov (United States)

    Valenzise, Mariella; Aversa, Tommaso; Salzano, Giuseppina; Zirilli, Giuseppina; De Luca, Filippo; Su, Maureen

    2017-01-19

    Autoimmune polyendocrinopathy-candidiasis-ectodermal-dystrophy (APECED) is a rare primary immunodeficiency disorder typically caused by homozygous AIRE gene mutation. It is characterized by the association of multiple autoimmune diseases, with a classical triad including chronic mucocutaneous candidiasis, hypoparathyroidism and adrenocortical failure. Its clinical spectrum has significantly enlarged in the last years with the apparence of new entities. One of these novel manifestations is the chronic inflammatory demyelinating polineuropathy (CIDP), that is characterized by involvement of peripheral nervous system, with nerve demyelination, progressive muscular weakness of both arms and legs and sensory loss. The identification of myelin protein zero as an important autoantigen (Ag) in CIDP may suggest the development of Ag-based therapies, such as Ag-specific DNA vaccination or infusion of Ag-coupled cells.

  20. Non-HTLV-I associated pleomorphic T-cell lymphoma of the brain mimicking post-vaccinal acute inflammatory demyelination.

    Science.gov (United States)

    Wanschitz, J; Hainfellner, J A; Simonitsch, I; Schnizer, M; Deisenhammer, E; Terunuma, H; Iwasaki, Y; Budka, H

    1997-02-01

    Two weeks after vaccination against tick-borne encephalitis (TBE), a 57-year-old female suddenly developed mental confusion and hemiparesis of the left side. Cranial MRI demonstrated extensive bilateral lesions in the fronto-parietal white matter of both hemispheres, suggesting an acute inflammatory demyelinating disease following vaccination. Despite administration of high-dose corticosteroids, the patient died 3 weeks after onset of neurological symptoms. Autopsy revealed diffuse infiltrates of a primary cerebral pleomorphic T-cell lymphoma of medium and large cell type. PCR on brain tissue for HTLV-I and serology for anti-HTLV-I antibodies in CSF and serum were negative; immunocytochemistry on brain tissue did not detect EBV-related antigen. This is the first recorded observation of a diffusely infiltrating primary central nervous system T-cell lymphoma, clinically and radiologically mimicking a fatal acute inflammatory demyelinating complication after vaccination.

  1. Treatment of Chronic Inflammatory Demyelinating Polyneuropathy: From Molecular Bases to Practical Considerations

    Directory of Open Access Journals (Sweden)

    Paolo Ripellino

    2014-01-01

    Full Text Available Chronic inflammatory demyelinating polyneuropathy (CIDP is an autoimmune disease of the peripheral nervous system, in which both cellular and humoral immune responses are involved. The disease is clinically heterogeneous with some patients displaying pure motor form and others also showing a variable degree of sensory dysfunction; disease evolution may also differ from patient to patient, since monophasic, progressive, and relapsing forms are reported. Underlying such clinical variability there is probably a broad spectrum of molecular dysfunctions that are and will be the target of therapeutic strategies. In this review we first explore the biological bases of current treatments and subsequently we focus on the practical management that must also take into account pharmacoeconomic issues.

  2. Case report: acute demyelinating encephalomyelitis following viper bite.

    Science.gov (United States)

    Xu, Anyi; Shan, Renfei; Huang, Daochao; Zhou, Jiajia; Keenoo, Anaswasseem; Qin, Jie

    2016-11-01

    The most serious complications of the central nervous system that occur after venomous snake bite are intracranial hemorrhage and ischemic stroke.We present a rarely seen central nervous system complication, acute demyelinating encephalomyelitis, after a treated Deinagkistrodon's viper bite.On April 5, 2015, a 50-year-old male farmer was bitten on his right leg by a Deinagkistrodon's viper. The bite rendered the victim unconscious for 14 days, during which he was treated with tetanus toxoid and polyvalent antisnake venom. Acute demyelinating encephalomyelitis (ADEM) was suspected after magnetic resonance imaging of the brain. After a high dose of methylprednisolone was used as diagnostic treatment, the patient started recovering fast.ADEM is a rare complication after snake bite, and is triggered by venom or antivenin. Magnetic resonance imaging helps in the early diagnosis of ADEM, and high-dose corticosteroid therapy appears to be effective in ADEM after viper bite or antivenin management.

  3. Isolated paroxysmal dysarthria caused by a single demyelinating midbrain lesion.

    Science.gov (United States)

    Codeluppi, Luca; Bigliardi, Guido; Chiari, Annalisa; Meletti, Stefano

    2013-10-16

    Paroxysmal dysarthria is an unusual condition characterised by brief episodes of dysarthria with the sudden onset and frequent recurrence. It has been mainly reported in multiple sclerosis and an association with midbrain lesions has been claimed; however, most of the reported patients had multiple brain alterations so it was difficult to associate this symptom with a specific lesion site. We illustrate the cases of two patients with an isolated demyelinating midbrain lesion presenting paroxysmal dysarthria as the only symptom; both participants had oligoclonal bands in the cerebrospinal fluid and an unremarkable follow-up. Both patients had benefit from carbamazepine treatment, similarly to previously reported cases. Our report confirms that a demyelinating midbrain lesion is sufficient to provoke paroxysmal dysarthria. It is noteworthy that an erroneous diagnosis of psychogenic disorders was initially made in both cases, highlighting the importance not to underestimate isolated paroxysmal symptoms in clinical practice.

  4. [Proteomic technologies for identification of serum potential biomarkers of autoimmune demyelinating polyneuropathies].

    Science.gov (United States)

    Ziganshin, R Kh; Arapidi, G P; Azarkin, I V; Balmasova, I P; Timchenko, O L; Fed'kina, Iu A; Morozova, E A; Piradov, M A; Suponeva, N A; Iushchuk, N D; Govorun, V M

    2011-01-01

    Time-of-flight MALDI mass spectrometry (MALDI-TOF-MS) profiling of blood serum of patients with Guillain-Barré syndrome (GBS, 36 samples), chronic inflammatory demyelinating polyneuropathy (CIDP, 24 samples) and practically healthy donors (HD) (35 samples) was carried out in order to identify potential biomarkers of autoimmune demyelinating polyneuropathies (ADP). To simplify the peptide-protein mixture of serum prior to MALDI-TOF-MS analysis samples were pre-fractionated on magnetic microparticles with a weak cation-exchange (MB-WCX) surface. Comparative analysis of mass spectrometric data using the classification algorithms (genetic and neural network-controlled) revealed a characteristic set of peaks, agreed change area with a high specificity and sensitivity of the differentiated mass spectrometry profiles of the blood serum of patients with DPNP and healthy donors (for GBS values of these characteristics reached 100 and 100, and for CIDP 94.1 and 100% respectively). Comparative analysis of mass spectrometric profiles of serum samples obtained from patients with GBS and CIDP, allowed to build a classification model to differentiate these diseases from each other, with a specificity of 88.9 and a sensitivity of 80%.

  5. Focal Hand Dystonia as a Sign of Demyelinating Attack in Multipl Sclerosis: 'Report of Three Cases’

    Directory of Open Access Journals (Sweden)

    Özge Öcek

    2014-12-01

    Full Text Available Although it is known that dystonia is a basal ganglia disease, dystonic symptoms have been observed in association with lesions of various sites located in sensory and motor pathways. We report three cases of paroxysmal focal hand dystonia, which may be due to the damage of the somatosensorial pathways in the cervical spinal cord. We suggest that the dystonia in our patients may be related to these active demyelinating cervical plaques. Two female and one male patients with definite relapsing remitting MS between the ages of 22 to 45 were admitted with serious disability while using their right hands. In all three cases abnormal posture in the right hand and involuntary sustained contractions together with minor choreiform movements of the fingers were observed. Cervical MRI showed contrast-enhancing demyelinating lesions at the level of C2-3 in all. In one of the patient’s cranial MRI revealed also two new contrast-enhancing plaques on the neighbourhood of right posterior lateral ventricle and parietal cortex. No new or enhancing lesion was detected in the basal ganglia; indicating that the cervical spinal cord lesions were responsible for hand dystonia. In one of the patients, the right median SEP response was absent in accordance with the clinical symptom. All three patients were treated with 1 gram intravenous methylprednisolone a day for 5-10 days. Approximately one month later clinical symptoms have been completely disappeared and control cervical MRI revealed resolution of the active lesions in all.

  6. Elevated leukocyte count in cerebrospinal fluid of patients with chronic inflammatory demyelinating polyneuropathy.

    Science.gov (United States)

    Lucke, Ilse M; Peric, Stojan; van Lieverloo, Gwen G A; Wieske, Luuk; Verhamme, Camiel; van Schaik, Ivo N; Basta, Ivana; Eftimov, Filip

    2018-01-17

    Cerebrospinal fluid (CSF) examination is often part of the diagnostic work-up of a patient suspected of having chronic inflammatory demyelinating polyneuropathy (CIDP). According to the European Federation of Neurological Societies and the Peripheral Nerve Society (EFNS/PNS) criteria, an elevated protein level without pleocytosis (leukocytes leukocytes are compatible with the diagnosis CIDP and how extensive the diagnostic work-up should be in patients with a demyelinating neuropathy and pleocytosis. We performed a retrospective study at two tertiary neuromuscular referral clinics and identified 14 out of 273 (6%) patients with CIDP with elevated CSF leukocytes (≥10 cells/µl). All these patients met the EFNS/PNS criteria for definite or probable CIDP. Eight patients (57%) presented with a subacute onset and four patients with an antecedent infection. Most patients responded well to therapy, and eight patients are currently in remission. In four patients, lumbar puncture was repeated. A spontaneous decrease in leukocytes before start of treatment was found in three patients. Our data indicate that a mild to moderate pleocytosis in CSF does not exclude the diagnosis of CIDP, especially in patients with a subacute onset of disease. © 2018 Peripheral Nerve Society.

  7. Quantitative muscle ultrasound is useful for evaluating secondary axonal degeneration in chronic inflammatory demyelinating polyneuropathy.

    Science.gov (United States)

    Hokkoku, Keiichi; Matsukura, Kiyoshi; Uchida, Yudai; Kuwabara, Midori; Furukawa, Yuichi; Tsukamoto, Hiroshi; Hatanaka, Yuki; Sonoo, Masahiro

    2017-10-01

    In chronic inflammatory demyelinating polyneuropathy (CIDP), exclusion of secondary axonal degeneration is challenging with conventional methods such as nerve conduction study (NCS), needle electromyography, and nerve biopsy. Increased echo intensity (EI) and decreased muscle thickness (MT) identified on muscle ultrasound (MUS) examination represent muscle denervation due to axonal degeneration in neurogenic disorders, suggesting MUS as a new tool to detect secondary axonal degeneration in patients with CIDP. EI and MT of abductor pollicis brevis, abductor digiti minimi, and first dorsal interosseous muscles were measured in 16 CIDP patients. Raw values were converted into z -scores using data from 60 normal controls (NCs). Six of 45 muscles showed abnormally high EI and low MT, suggesting denervation following secondary axonal degeneration. These six muscles belonged to two patients with long disease history, unresponsiveness to treatment, and long interval from onset to initial therapy. There were no significant differences in EI and MT ( p  = .23 and .67, respectively) between the CIDP and NC groups, although NCS results revealed obvious demyelinating abnormalities in all CIDP patients, suggesting the fact that muscle structures will be preserved, and EI and MT will not change unless secondary axonal degeneration occurs in CIDP. MUS is a promising tool for evaluating secondary axonal degeneration in patients with CIDP.

  8. Recurrent angio-fibroma of breast masquerading as phyllodes tumor.

    Science.gov (United States)

    Chaurasia, Jai K; Alam, Feroz; Shadan, Mariam; Naim, Mohammed

    2015-01-01

    A young Indian female presented with a recurring tumor in the right breast masquerading as phyllodes tumor. Patient had history of five times excision and recurrences of the tumor, diagnosed as fibrous phyllodes of the breast. Presently, a well-circumscribed tumor of about 10 cm size, comprising of benign fibrous-angiomatous tissue with evidence of foci of pyogenic vasculitis was observed. Immuno-histochemical markers for the myo-epithelial and epithelial elements excluded the possibility of fibrous phyllodes, inflammatory myofibroblastic tumor, desmoid fibromatosis, and metaplastic carcinoma. The present findings were diagnostic of an inflammatory angio-fibroma of the right breast, not reported in the earlier literature. The observations indicated that the female breast may be susceptible to spontaneous productive and common-antibiotic-resistant focal septic vascular inflammation giving rise to angio-fibromatous proliferation producing a well-defined tumor mass in the breast, distinguishable from the other breast lesions by the connective tissue stains and immuno-histochemical markers.

  9. Appendiceal Diverticulitis Clinically Masquerading as an Appendiceal Carcinoma

    Directory of Open Access Journals (Sweden)

    Tadashi Terada

    2014-01-01

    Full Text Available Appendiceal diverticulosis is a rare condition. Herein reported is a case of appendiceal diverticulosis and diverticulitis clinically masquerading as appendiceal carcinoma. A 62-year-old woman presented with abdominal pain. US and CT showed a tumor measuring 5 × 4 × 4 cm in vermiform appendix. Colon endoscopy showed mucosal elevation and irregularity in the orifice of vermiform appendix. A biopsy of the appendiceal mucosa showed no significant changes. Clinical diagnosis was appendiceal carcinoma and wide excision of terminal ileum, appendix, cecum, and ascending colon was performed. Grossly, the appendix showed a tumor measuring 5 × 3 × 4 cm. The appendiceal lumen was opened, and the appendiceal mucosa was elevated and irregular. The periappendiceal tissue showed thickening. Microscopically, the lesion was multiple appendiceal diverticula. The diverticula were penetrating the muscle layer. The mucosa showed erosions in places. Much fibrosis, abscess formations, and lymphocytic infiltration were seen in the subserosa. Abscesses were also seen in the diverticular lumens. Some diverticula penetrated into the subserosa. The pathologic diagnosis was appendiceal diverticulitis. When they encounter an appendiceal mass, clinicians should consider appendiceal diverticulitis as a differential diagnosis.

  10. Antibody-induced generation of reactive oxygen radicals by brain macrophages in canine distemper encephalitis: a mechanism for bystander demyelination.

    Science.gov (United States)

    Griot, C; Bürge, T; Vandevelde, M; Peterhans, E

    1989-01-01

    The mechanism of inflammatory demyelination in canine distemper encephalitis (CDE) is uncertain but macrophages are thought to play an important effector role in this lesion. Serum and cerebrospinal fluid (CSF), containing anti-canine distemper virus and anti-myelin antibodies from dogs with CDE were tested for their ability to generate reactive oxygen species (ROS) in macrophages in primary dog brain cell cultures using a chemiluminescence (CL) assay. The majority of serum samples and several CSF samples from animals with inflammatory demyelination elicited a CL signal in infected dog brain cell cultures. In contrast, none of these samples induced a positive response in uninfected cultures which contained large numbers of myelin antigen-presenting cells, although defined anti-myelin antibodies lead to a marked secretion of ROS in this system. It was concluded that antiviral antibody-induced secretion of ROS, known to be highly toxic for brain tissue, may play an important role in white matter damage in inflammatory lesions supporting a previous hypothesis of bystander demyelination in CDE. No evidence was found for a similar antibody-dependent cellular cytotoxicity-like mechanism mediated by anti-myelin antibodies in CDE, which does not support the concept of autoimmunity in this disease.

  11. Neurotropin® Accelerates the Differentiation of Schwann Cells and Remyelination in a Rat Lysophosphatidylcholine-Induced Demyelination Model

    Directory of Open Access Journals (Sweden)

    Hozo Matsuoka

    2018-02-01

    Full Text Available Neurotropin® (NTP, a non-protein extract of inflamed rabbit skin inoculated with vaccinia virus, is clinically used for the treatment of neuropathic pain in Japan and China, although its effect on peripheral nerve regeneration remains to be elucidated. The purpose of this study was to investigate the effects of NTP on Schwann cells (SCs in vitro and in vivo, which play an important role in peripheral nerve regeneration. In SCs, NTP upregulated protein kinase B (AKT activity and Krox20 and downregulated extracellular signal-regulated kinase1/2 activity under both growth and differentiation conditions, enhanced the expression of myelin basic protein and protein zero under the differentiation condition. In a co-culture of dorsal root ganglion neurons and SCs, NTP accelerated myelination of SCs. To further investigate the influence of NTP on SCs in vivo, lysophosphatidylcholine was injected into the rat sciatic nerve, leading to the focal demyelination. After demyelination, NTP was administered systemically with an osmotic pump for one week. NTP improved the ratio of myelinated axons and motor, sensory, and electrophysiological function. These findings reveal novel effects of NTP on SCs differentiation in vitro and in vivo, and indicate NTP as a promising treatment option for peripheral nerve injuries and demyelinating diseases.

  12. Mice lacking Gpr37 exhibit decreased expression of the myelin-associated glycoprotein MAG and increased susceptibility to demyelination.

    Science.gov (United States)

    Smith, Brilee M; Giddens, Michelle M; Neil, Jessica; Owino, Sharon; Nguyen, TrangKimberly T; Duong, Duc; Li, Fengqiao; Hall, Randy A

    2017-09-01

    GPR37 is an orphan G protein-coupled receptor that is predominantly expressed in the brain and found at particularly high levels in oligodendrocytes. GPR37 has been shown to exert effects on oligodendrocyte differentiation and myelination during development, but the molecular basis of these actions is incompletely understood and moreover nothing is known about the potential role(s) of this receptor under demyelinating conditions. To shed light on the fundamental biology of GPR37, we performed proteomic studies comparing protein expression levels in the brains of mice lacking GPR37 and its close relative GPR37-like 1 (GPR37L1). These studies revealed that one of the proteins most sharply decreased in the brains of Gpr37/Gpr37L1 double knockout mice is the myelin-associated glycoprotein MAG. Follow-up Western blot studies confirmed this finding and demonstrated that genetic deletion of Gpr37, but not Gpr37L1, results in strikingly decreased brain expression of MAG. Further in vitro studies demonstrated that GPR37 and MAG form a complex when expressed together in cells. As loss of MAG has previously been shown to result in increased susceptibility to brain insults, we additionally assessed Gpr37-knockout (Gpr37 - / - ) vs. wild-type mice in the cuprizone model of demyelination. These studies revealed that Gpr37 - / - mice exhibit dramatically increased loss of myelin in response to cuprizone, yet do not show any increased loss of oligodendrocyte precursor cells or mature oligodendrocytes. These findings reveal that loss of GPR37 alters oligodendrocyte physiology and increases susceptibility to demyelination, indicating that GPR37 could be a potential drug target for the treatment of demyelinating diseases such as multiple sclerosis. Copyright © 2017 IBRO. Published by Elsevier Ltd. All rights reserved.

  13. Burkitt's lymphoma masquerading as appendicitis--two case reports and review of the literature.

    Science.gov (United States)

    Weledji, Elroy P; Ngowe, Marcelin N; Abba, John S

    2014-06-18

    Two cases of Burkitt's lymphoma masquerading as appendicitis are reported herein. The diagnoses were made post-operatively from the appendix specimen in one case and from an ileocecal resection specimen for cecal fistula complicating an appendicectomy in the second case. These cases highlight the importance of routine histological examination of appendicectomy specimens.

  14. The Many Guises of Endometriosis: Giant Abdominal Wall Endometriosis Masquerading as An Incisional Hernia

    Directory of Open Access Journals (Sweden)

    Chiara Petrosellini

    2017-10-01

    Full Text Available Endometriosis is defined by the presence of ectopic endometrial tissue outside the uterine cavity. Although it is a leading cause of chronic pelvic pain and infertility, its clinical presentation can vary, resulting in diagnostic and therapeutic challenges. Extrapelvic endometriosis is particularly difficult to diagnose owing to its ability to mimic other conditions. Endometrial tissue in a surgical scar is uncommon and often misdiagnosed as a granuloma, abscess, or malignancy. Cyclical hemorrhagic ascites due to peritoneal endometriosis is exceptionally rare. We report the case of a pre-menopausal, nulliparous 44-year-old woman who presented with ascites and a large abdominal mass that arose from the site of a lower midline laparotomy scar. Five years previously, she had undergone open myomectomy for uterine fibroids. Soon after her initial operation she developed abdominal ascites, which necessitated percutaneous drainage on multiple occasions. We performed a laparotomy with excision of the abdominal wall mass through an inverted T incision. The extra-abdominal mass consisted of mixed cystic and solid components, and weighed 1.52 kg. It communicated with the abdominopelvic cavity through a 2 cm defect in the linea alba. The abdomen contained a large amount of odourless, brown fluid which drained into the mass. There was a large capsule that covered the small and large bowel, liver, gallbladder, and stomach. Final histology reported a 28×19×5 cm mass of endometrial tissue with no evidence of malignant transformation. The patient recovered well post-operatively and has remained asymptomatic. Our case illustrates that, despite being a common disease, endometriosis can masquerade as several other conditions and be missed or diagnosed late. Delay in diagnosis will not only prolong symptoms but can also compromise reproductive lifespan. It is therefore paramount that endometriosis is to be considered early in the management of premenopausal women

  15. Increased severity of experimental autoimmune encephalomyelitis, chronic macrophage/microglial reactivity, and demyelination in transgenic mice producing tumor necrosis factor-alpha in the central nervous system

    DEFF Research Database (Denmark)

    Taupin, V; Renno, T; Bourbonnière, L

    1997-01-01

    Tumor necrosis factor-alpha (TNF-alpha) is an inflammatory cytokine implicated in a number of autoimmune diseases. Apoptotic cell death is induced by TNF-alpha in vitro, and has been suggested as one cause of autoimmune pathology, including autoimmune demyelinating diseases where oligodendrocytes...... are a target of immune attack. TNF-alpha also regulates macrophage activity which could contribute to autoimmune inflammation. We have expressed TNF-alpha at disease-equivalent levels in the central nervous system of transgenic mice, using a myelin basic protein (MBP) promoter. These mice were normal...... and showed no spontaneous pathology, but they developed experimental autoimmune encephalomyelitis (EAE) with greater severity than nontransgenic controls when immunized with MBP in adjuvant. Unlike nontransgenic controls, EAE then progressed to a nonabating demyelinating disease. Macrophage...

  16. Unilateral submandibular gland aplasia masquerading as cancer nodal metastasis.

    Science.gov (United States)

    Shipchandler, Taha Z; Lorenz, Robert R

    2008-01-01

    Five reports have examined unilateral submandibular gland aplasia. The purposes of this report are to demonstrate submandibular gland aplasia leading to contralateral gland hypertrophy in the setting of oral cavity cancer and to discuss the corresponding diagnostic and management challenges. This study is a case report of a 60-year-old male who presented with pain on the right side of the mobile tongue. This report uses literature review. A 60-year-old male presented with pain on the right side of the mobile tongue. Subsequent results of punch biopsy revealed squamous cell carcinoma in situ with foci of microinvasion of the tongue. Head and neck examination revealed no abnormalities. The patient underwent a wide-local excision of the tongue lesion. Postoperative computed tomographic (CT) scan showed an asymmetric mass on the ipsilateral side of the cancer in the region of the submandibular gland. The gland was noted to be abnormally large. A diagnosis of contralateral submandibular gland aplasia was made. The patient is cancer-free at 2 years postlocal excision. Salivary gland aplasia is an extremely rare disorder and is often associated with various congenital syndromes. Unilateral submandibular gland aplasia is even rarer with ours representing the sixth reported case. Aplasia is believed to stem from a regional disturbance in early fetal development. Common symptoms can include dysphagia, dry mouth, decreased taste, and tooth decay. In the presence of a history of oral cavity cancer, unilateral submandibular gland aplasia poses a challenge during postoperative cancer follow-up. Unilateral submandibular gland aplasia in the setting of oral cavity cancer poses a unique challenge for cancer follow-up. Hypertrophy of the submandibular gland on the other side can masquerade as nodal metastasis. Head and neck examination as well as CT scan can be inconclusive. Regular confirmatory tests such as fine needle aspiration biopsy and positron emission tomography/CT for

  17. IFN-beta gene deletion leads to augmented and chronic demyelinating experimental autoimmune encephalomyelitis

    DEFF Research Database (Denmark)

    Teige, Ingrid; Treschow, Alexandra; Teige, Anna

    2003-01-01

    -beta knockout (KO) mice are more susceptible to EAE than their wild-type (wt) littermates; they develop more severe and chronic neurological symptoms with more extensive CNS inflammation and demyelination. However, there was no discrepancy observed between wt and KO mice regarding the capacity of T cells...... to proliferate or produce IFN-gamma in response to recall Ag. Consequently, we addressed the effect of IFN-beta on encephalitogenic T cell development and the disease initiation phase by passive transfer of autoreactive T cells from KO or wt littermates to both groups of mice. Interestingly, IFN-beta KO mice...... microglia activation, resulting in a sustained inflammation, cytokine production, and tissue damage with consequent chronic neurological deficits....

  18. Fibroblast growth factor signaling in oligodendrocyte-lineage cells facilitates recovery of chronically demyelinated lesions but is redundant in acute lesions.

    Science.gov (United States)

    Furusho, Miki; Roulois, Aude J; Franklin, Robin J M; Bansal, Rashmi

    2015-10-01

    Remyelination is a potent regenerative process in demyelinating diseases, such as multiple sclerosis, the effective therapeutic promotion of which will fill an unmet clinical need. The development of proregenerative therapies requires the identification of key regulatory targets that are likely to be involved in the integration of multiple signaling mechanisms. Fibroblast growth factor (FGF) signaling system, which comprises multiple ligands and receptors, potentially provides one such target. Since the FGF/FGF receptor (FGFR) interactions are complex and regulate multiple diverse functions of oligodendrocyte lineage cells, it is difficult to predict their overall therapeutic potential in the regeneration of oligodendrocytes and myelin. Therefore, to assess the integrated effects of FGFR signaling on this process, we simultaneously inactivated both FGFR1 and FGFR2 in oligodendrocytes and their precursors using two Cre-driver mouse lines. Acute and chronic cuprizone-induced or lysolecithin-induced demyelination was established in Fgfr1/Fgfr2 double knockout mice (dKO). We found that in the acute cuprizone model, there was normal differentiation of oligodendrocytes and recovery of myelin in the corpus callosum of both control and dKO mice. Similarly, in the spinal cord, lysolecithin-induced demyelinated lesions regenerated similarly in the dKO and control mice. In contrast, in the chronic cuprizone model, fewer differentiated oligodendrocytes and less efficient myelin recovery were observed in the dKO compared to control mice. These data suggest that while cell-autonomous FGF signaling is redundant during recovery of acute demyelinated lesions, it facilitates regenerative processes in chronic demyelination. Thus, FGF-based therapies have potential value in stimulating oligodendrocyte and myelin regeneration in late-stage disease. © 2015 Wiley Periodicals, Inc.

  19. Alemtuzumab in the treatment of IVIG-dependent chronic inflammatory demyelinating polyneuropathy.

    LENUS (Irish Health Repository)

    Marsh, E A

    2010-06-01

    Chronic inflammatory demyelinating polyneuropathy (CIDP) is an idiopathic immune mediated neuropathy causing demyelination and conduction block thought to occur as the result of an aberrant autoimmune response resulting in peripheral nerve inflammation mediated by T cells and humoral factors. Diagnosis commonly prompts initial treatment with steroids or intravenous immunoglobulin (IVIG) on which 5-35% subsequently become dependent to maintain function. Despite a number of small scale trials, the role for alternative long-term immunosuppression remains unclear. Alemtuzumab is a humanised monoclonal antibody targeting the CD52 antigen present on the surface of lymphocytes and monocytes. A single intravenous infusion results in rapid and profound lymphopoenia lasting >12 months. We report its use and clinical outcome in a small series of patients with severe IVIG-dependent CIDP. Seven patients (4 Males; 3 Females) who had failed to respond to conventional immunosuppression were treated in 5 centres receiving 9 courses of alemtuzumab (dose range 60-150 mg). Following treatment, mean monthly IVIG use fell 26% from 202 to 149 g and IVIG administration frequency from 22 to 136 days. Two patients had prolonged remission, two patients had a partial response and no clear benefit was observed in the remaining three patients (2 Males, 1 Females). Responding patients had a younger age at onset (19.5 years) and shorter disease duration than non-responders. Three patients developed autoimmune disease following treatment. Alemtuzumab may offer an alternative treatment for a subset of early onset IVIG dependent CIDP patients failing conventional immunosuppressive agents, but concerns about toxicity may limit its use.

  20. Myelin oligodendrocyte glycoprotein and aquaporin-4 antibodies are highly specific in children with acquired demyelinating syndromes.

    Science.gov (United States)

    Duignan, Sophie; Wright, Sukhvir; Rossor, Tom; Cazabon, John; Gilmour, Kimberly; Ciccarelli, Olga; Wassmer, Evangeline; Lim, Ming; Hemingway, Cheryl; Hacohen, Yael

    2018-02-22

    Our objectives were to evaluate the utility of measuring myelin oligodendrocyte glycoprotein (MOG) and aquaporin-4 (AQP4) antibodies (Ab) in clinical practice and describe their associated neurological phenotypes in children. Between 2012 and 2017, 371 children with suspected acquired demyelinating syndromes (ADS) seen in three tertiary centres were tested for MOG-Ab and AQP4-Ab. Medical notes were retrospectively reviewed, and clinical and demographic data compiled. Clinical phenotyping was performed blinded to the antibody results. After review, 237 of the 371 were diagnosed with ADS. Of these, 76 out of 237 (32.1%) were MOG-Ab positive and 14 out of 237 (5.9%) were AQP4-Ab positive. None were positive for both autoantibodies. All 134 patients with non-ADS were negative for MOG-Ab. MOG-Ab were identified in 45 out of 70 (64.3%) patients presenting with acute disseminated encephalomyelitis (ADEM) and in 24 out of 25 patients with relapsing ADEM. Thirty-six out of 75 (48%) MOG-Ab positive patients relapsed. Of the 33 children with neuromyelitis optic spectrum disorder, 14 were AQP4-Ab positive, 13 were MOG-Ab positive, and 6 were seronegative. Of the children with longitudinal samples, 8 out of 13 AQP4-Ab remained positive during the disease course compared to 35 out of 43 MOG-Ab (13/16 monophasic and 22/27 relapsing). Myelin oligodendrocyte glycoprotein antibodies were identified in a third of children with ADS. Almost half of the MOG-Ab positive children relapsed and the majority of them remained antibody positive over 4-years follow-up. Myelin oligodendrocyte glycoprotein antibodies (MOG-Ab) are highly specific for acquired demyelinating syndromes (ADS). Myelin oligodendrocyte glycoprotein antibodies are not identified in children with peripheral demyelination or genetic leukodystrophies/hypomyelination. Up to 48% of MOG-Ab ADS paediatric patients relapse, higher than previously thought. Seroconversion to MOG-Ab negative status is infrequent; patients may test

  1. The effect of irradiation on demyelination induced by a virulent Semliki Forest virus

    International Nuclear Information System (INIS)

    Chew-Lim, M.; Webb, H.E.; Jagelma, S.

    1977-01-01

    A dose of 500 rad total body irradiation before Semliki Forest virus infection was the most effective in producing multiple foci of demyelination in Swiss mice. Animals receiving this dose had the highest virus titre of Semliki Forest virus persisting in the brain and a delayed antibody response. In spite of extensive demyelination no obvious clinical signs such as paralysis were observed. (author)

  2. Immunological Demyelination Triggers Macrophage/Microglial Cells Activation without Inducing Astrogliosis

    Directory of Open Access Journals (Sweden)

    Frank Cloutier

    2013-01-01

    Full Text Available The glial scar formed by reactive astrocytes and axon growth inhibitors associated with myelin play important roles in the failure of axonal regeneration following central nervous system (CNS injury. Our laboratory has previously demonstrated that immunological demyelination of the CNS facilitates regeneration of severed axons following spinal cord injury. In the present study, we evaluate whether immunological demyelination is accompanied with astrogliosis. We compared the astrogliosis and macrophage/microglial cell responses 7 days after either immunological demyelination or a stab injury to the dorsal funiculus. Both lesions induced a strong activated macrophage/microglial cells response which was significantly higher within regions of immunological demyelination. However, immunological demyelination regions were not accompanied by astrogliosis compared to stab injury that induced astrogliosis which extended several millimeters above and below the lesions, evidenced by astroglial hypertrophy, formation of a glial scar, and upregulation of intermediate filaments glial fibrillary acidic protein (GFAP. Moreover, a stab or a hemisection lesion directly within immunological demyelination regions did not induced astrogliosis within the immunological demyelination region. These results suggest that immunological demyelination creates a unique environment in which astrocytes do not form a glial scar and provides a unique model to understand the putative interaction between astrocytes and activated macrophage/microglial cells.

  3. Central nervous system demyelination associated with Etanercept in a 51 year old woman

    Directory of Open Access Journals (Sweden)

    Dirik Dileu

    2007-03-01

    Full Text Available There are few case reports documenting a new onset of demyelinating processes in patients receiving anti-tumour necrosis factor alpha therapy (anti-TNF alpha for chronic inflammatory arthropathies. Whether anti-TNF alpha therapy induces new onset demyelination or just exacerbates pre-existing latent multiple sclerosis is not fully understood. We are reporting a 51-year-old woman without a prior history of multiple sclerosis, who developed demyelinating brain lesions three months after starting Etanercept. Her symptoms partially resolved on cessation of the drug. Our case was unusual compared to some previous case reports, as the patient’s age at presentation was beyond that for idiopathic multiple sclerosis. This may strengthen the hypothesis of a causal relationship between new onset demyelination and Etanercept; however, exacerbation of pre-existing demyelinating process by Etanercept in this patient still cannot be totally excluded. We recommend doing magnetic resonance imaging (MRI of the brain before starting patients on anti-TNF alpha therapy to exclude latent demyelination. In addition, new onset demyelination following anti-TNF alpha therapy should be reported and studied thoroughly as this may yield a significant advancement in our understanding of the pathogenesis of multiple sclerosis. Long-term follow-up of these cases is also important to determine the long-term prognosis and the rate of relapse of demyelinating process in this group of patients.

  4. Simulated auditory nerve axon demyelination alters sensitivity and response timing to extracellular stimulation.

    Science.gov (United States)

    Resnick, Jesse M; O'Brien, Gabrielle E; Rubinstein, Jay T

    2018-04-01

    Since cochlear implant function involves direct depolarization of spiral ganglion neurons (SGNs) by applied current, SGN physiological health must be an important factor in cochlear implant (CI) outcomes. This expected relationship has, however, been difficult to confirm in implant recipients. Suggestively, animal studies have demonstrated both acute and progressive SGN ultrastructural changes (notably axon demyelination), even in the absence of soma death, and corresponding altered physiology following sensorineural deafening. Whether such demyelination occurs in humans and how such changes might impact CI function remains unknown. To approach this problem, we incorporated SGN demyelination into a biophysical model of extracellular stimulation of SGN fibers. Our approach enabled exploration of the entire parameter space corresponding to simulated myelin diameter and extent of fiber affected. All simulated fibers were stimulated distally with anodic monophasic, cathodic monophasic, anode-phase-first (AF) biphasic, and cathode-phase-first (CF) biphasic pulses from an extracellular disc electrode and monitored for spikes centrally. Not surprisingly, axon sensitivity generally decreased with demyelination, resulting in elevated thresholds, however, this effect was strongly non-uniform. Fibers with severe demyelination affecting only the most peripheral nodes responded nearly identically to normally myelinated fibers. Additionally, partial demyelination (<50%) yielded only minimal increases in threshold even when the entire fiber was impacted. The temporal effects of demyelination were more unexpected. Both latency and jitter of responses demonstrated resilience to modest changes but exhibited strongly non-monotonic and stimulus-dependent relationships to more profound demyelination. Normal, and modestly demyelinated fibers, were more sensitive to cathodic than anodic monophasic pulses and to CF than AF biphasic pulses, however, when demyelination was more severe these

  5. Neurochemical and neuroendocrine correlates of overactive bladder at first demyelinating episode.

    Science.gov (United States)

    Koutsis, Georgios; Evangelopoulos, Maria-Eleftheria; Sfagos, Constantinos; Markianos, Manolis

    2016-11-01

    Bladder dysfunction is frequent during the course of multiple sclerosis (MS), observed in up to 75% of patients. Urinary symptomatology can be a feature of the first episode of MS in a minority of cases, and most often shows characteristics of an overactive bladder (OAB), with voiding symptoms seen less frequently, often in combination with OAB. The neural control of micturition is complex, involving systems located in the brain, spinal cord, and periphery, and implicating central noradrenergic, serotonergic, and dopaminergic activities. Urinary disorders are also linked to anxiety and depression, conditions connected to hypothalamus-pituitary-adrenal axis activity. In this study we aimed to investigate neurochemical and neuroendocrine correlates of bladder dysfunction in early MS. We included 101 patients at first demyelinating episode suggestive of MS that were drug-free at assessment. We evaluated the presence of urinary symptomatology and estimated CSF levels of the main metabolites of noradrenaline, serotonin, and dopamine, as well CSF-ACTH and serum cortisol. In total, 15 patients (15%) reported urinary dysfunction suggestive of OAB. Four of these had coexistent voiding symptomatology. The serotonin metabolite 5-HIAA was significantly reduced (P = 0.017) in patients with OAB syndrome, while there were no differences in the metabolites of noradrenaline (MHPG) and of dopamine (HVA). Additionally, significantly lower serum cortisol (P = 0.009) and borderline lower CSF-ACTH (P = 0.08) were found in patients with OAB. MS patients with OAB syndrome at the first demyelinating episode show reductions in central serotonergic activity and stress hormones. Whether the same changes persist at later disease stages remains to be investigated. Neurourol. Urodynam. 35:955-958, 2016. © 2015 Wiley Periodicals, Inc. © 2015 Wiley Periodicals, Inc.

  6. Apoptosis of Oligodendrocytes during Early Development Delays Myelination and Impairs Subsequent Responses to Demyelination.

    Science.gov (United States)

    Caprariello, Andrew V; Batt, Courtney E; Zippe, Ingrid; Romito-DiGiacomo, Rita R; Karl, Molly; Miller, Robert H

    2015-10-14

    During mammalian development, myelin-forming oligodendrocytes are generated and axons ensheathed according to a tightly regulated sequence of events. Excess premyelinating oligodendrocytes are eliminated by apoptosis and the timing of the onset of myelination in any specific CNS region is highly reproducible. Although the developing CNS recovers more effectively than the adult CNS from similar insults, it is unknown whether early loss of oligodendrocyte lineage cells leads to long-term functional deficits. To directly assess whether the loss of oligodendrocytes during early postnatal spinal cord development impacted oligodendrogenesis, myelination, and remyelination, transgenic mouse lines were generated in which a modified caspase-9 molecule allowed spatial and temporal control of the apoptotic pathway specifically in mature, myelin basic protein expressing oligodendrocytes (MBP-iCP9). Activating apoptosis in MBP(+) cells of the developing spinal cord during the first postnatal week inhibited myelination. This inhibition was transient, and the levels of myelination largely returned to normal after 2 weeks. Despite robust developmental plasticity, MBP-iCP9-induced oligodendrocyte apoptosis compromised the rate and extent of adult remyelination. Remyelination failure correlated with a truncated proliferative response of oligodendrocyte progenitor cells, suggesting that depleting the oligodendrocyte pool during critical developmental periods compromises the regenerative response to subsequent demyelinating lesions. This manuscript demonstrates that early insults leading to oligodendrocyte apoptosis result in the impairment of recovery from demyelinating diseases in the adult. These studies begin to provide an initial understanding of the potential failure of recovery in insults, such as periventricular leukomalacia and multiple sclerosis. Copyright © 2015 the authors 0270-6474/15/3514031-11$15.00/0.

  7. Reported Changes in Dietary Behavior Following a First Clinical Diagnosis of Central Nervous System Demyelination

    Directory of Open Access Journals (Sweden)

    Rebecca D. Russell

    2018-03-01

    Full Text Available Background/objectivesAlthough the current evidence is insufficient to recommend a special diet for people with multiple sclerosis (MS, dietary advice for people with MS is prolific online and in the media. This study aimed to describe dietary changes made in the year following a first clinical diagnosis of central nervous system demyelination (FCD, a common precursor to MS.Subjects/methodsWe used follow-up data from the Ausimmune Study, a multicentre matched case-control study examining the environmental risk factors for a FCD. A total of 244 cases (60 male, 184 female completed a 1-year follow-up interview, which included a question about dietary changes. We described the number and proportion (% of participants who reported making dietary changes and the type of change made. We investigated independent predictors of making a dietary change using a multivariable logistic regression model.ResultsA total of 38% (n = 92 of participants at the 1-year follow-up reported making at least one dietary change over the last year. There were no statistically significant independent associations between any participant characteristic and odds of making a dietary change. Of those who made at least one dietary change, the most common changes were increasing fruit and/or vegetable intake (27%, n = 25 and following a low-fat diet (25%, n = 23.ConclusionA considerable proportion of the study population reported making at least one dietary change in the year following a FCD, with the majority of changes being toward a healthier diet. Further research is warranted to investigate the reasons behind any dietary changes adopted by people with a FCD or with MS, and whether making a dietary change has benefits for the progression of demyelinating diseases, e.g., to a diagnosis of MS, as well as for general health and well-being.

  8. Dysmyelination, demyelination and reactive astrogliosis in the optic nerve of the taiep rat

    Directory of Open Access Journals (Sweden)

    JUAN KRSULOVIC

    1999-01-01

    Full Text Available Taiep is an autosomal recessive mutant rat that shows a highly hypomyelinated central nervous system (CNS. Oligodendrocytes accumulate microtubules (MTs in association with endoplasmic reticulum (ER membranes forming MT-ER complexes. The microtubular defect in oligodendrocytes, the abnormal formation of CNS myelin and the astrocytic reaction were characterized by immunocytochemical and ultrastructural methods during the first year of life. Optic nerves of both control and taiep rats were processed by the immunoperoxidase method using antibodies against tubulin, myelin basic protein (MBP and glial fibrillary acidic protein (GFAP. Taiep oligodendrocytes are strongly immunoreactive against tubulin, indicative of a significant accumulation of microtubules. Early differentiated oligodendrocytes observed with electron microscopy show that MT-ER complexes are mainly present in the cell body. This defect increases during the first year of life; oligodendrocytes show large MT-ER complexes projected within oligodendrocyte processes. Using anti-MBP, there was a progressive reduction of immunolabeling in the myelin sheaths as taiep rats grew older. Ultrastructural analysis revealed severely dysmyelinated axons with a frequently collapsed periaxonal collar. However, through age the myelin sheath became gradually infiltrated by MTs, suggesting their contribution to premature loss of myelin in the taiep rat. Axons of one-year-old taiep rats were severely demyelinated. Modifications in astrocytes revealed by the GFAP antibody showed a strong hypertrophy with increased immunostaining in their processes. As demyelination of axons progressed, taiep rats developed a strong astrogliosis. The present findings suggest that in taiep rats the early abnormal myelination of axons affects the adequate maintenance of myelin, leading to a progressive loss of myelin components and severe astrogliosis, features that should be considered in the pathogenesis of dysmyelinating

  9. Astrogliosis During Acute and Chronic Cuprizone Demyelination and Implications for Remyelination

    Directory of Open Access Journals (Sweden)

    Norah Hibbits

    2012-10-01

    Full Text Available In multiple sclerosis, microglia/macrophage activation and astrocyte reactivity are important components of the lesion environment that can impact remyelination. The current study characterizes these glial populations relative to expression of candidate regulatory molecules in cuprizone demyelinated corpus callosum. Importantly, periods of recovery after acute or chronic cuprizone demyelination are examined to compare conditions of efficient versus limited remyelination, respectively. Microglial activation attenuates after early demyelination. In contrast, astrocyte reactivity persists throughout demyelination and a 6-week recovery period following either acute or chronic demyelination. This astrocyte reaction is characterized by (a early proliferation, (b increased expression of GFAP (glial fibrillary acidic protein, Vim (vimentin, Fn1 (fibronectin and CSPGs (chondroitin sulphate proteoglycans and (c elaboration of a dense network of processes. Glial processes elongated in the axonal plane persist throughout lesion areas during both the robust remyelination that follows acute demyelination and the partial remyelination that follows chronic demyelination. However, prolonged astrocyte reactivity with chronic cuprizone treatment does not progress to barrier formation, i.e. dense compaction of astrocyte processes to wall off the lesion area. Multiple candidate growth factors and inflammatory signals in the lesion environment show strong correlations with GFAP across the acute cuprizone demyelination and recovery time course, yet there is more divergence across the progression of chronic cuprizone demyelination and recovery. However, differential glial scar formation does not appear to be responsible for differential remyelination during recovery in the cuprizone model. The astrocyte phenotype and lesion characteristics in this demyelination model inform studies to identify triggers of non-remyelinating sclerosis in chronic multiple sclerosis

  10. Plasminogen Activator Inhibitor-1 Antagonist TM5484 Attenuates Demyelination and Axonal Degeneration in a Mice Model of Multiple Sclerosis.

    Directory of Open Access Journals (Sweden)

    Nicolas Pelisch

    Full Text Available Multiple sclerosis (MS is characterized by inflammatory demyelination and deposition of fibrinogen in the central nervous system (CNS. Elevated levels of a critical inhibitor of the mammalian fibrinolitic system, plasminogen activator inhibitor 1 (PAI-1 have been demonstrated in human and animal models of MS. In experimental studies that resemble neuroinflammatory disease, PAI-1 deficient mice display preserved neurological structure and function compared to wild type mice, suggesting a link between the fibrinolytic pathway and MS. We previously identified a series of PAI-1 inhibitors on the basis of the 3-dimensional structure of PAI-1 and on virtual screening. These compounds have been reported to provide a number of in vitro and in vivo benefits but none was tested in CNS disease models because of their limited capacity to penetrate the blood-brain barrier (BBB. The existing candidates were therefore optimized to obtain CNS-penetrant compounds. We performed an in vitro screening using a model of BBB and were able to identify a novel, low molecular PAI-1 inhibitor, TM5484, with the highest penetration ratio among all other candidates. Next, we tested the effects on inflammation and demyelination in an experimental allergic encephalomyelitis mice model. Results were compared to either fingolimod or 6α-methylprednisolone. Oral administration of TM5484 from the onset of signs, ameliorates paralysis, attenuated demyelination, and axonal degeneration in the spinal cord of mice. Furthermore, it modulated the expression of brain-derived neurotrophic factor, which plays a protective role in neurons against various pathological insults, and choline acetyltransferase, a marker of neuronal density. Taken together, these results demonstrate the potential benefits of a novel PAI-1 inhibitor, TM5484, in the treatment of MS.

  11. Laquinimod interferes with migratory capacity of T cells and reduces IL-17 levels, inflammatory demyelination and acute axonal damage in mice with experimental autoimmune encephalomyelitis.

    Science.gov (United States)

    Wegner, Christiane; Stadelmann, Christine; Pförtner, Ramona; Raymond, Emanuel; Feigelson, Sara; Alon, Ronen; Timan, Bracha; Hayardeny, Liat; Brück, Wolfgang

    2010-10-08

    We investigated the effect of laquinimod on inflammatory demyelination, axonal damage, cytokine profiles and migratory capacities of lymphocytes in C57BL/6 mice with active EAE induced with MOG(35-55) peptide. The mice were treated at disease induction and after disease onset. Spinal cords were assessed histologically. Cytokines and adhesive properties were analyzed in splenocytes. Preventive and therapeutic laquinimod treatment reduced clinical signs, inflammation, and demyelination. VLA-4-mediated adhesiveness and pro-inflammatory cytokines such as IL-17 were down-regulated in treated animals. Within lesions, treated mice showed similar axonal densities, but less acute axonal damage than controls. Laquinimod might thus protect myelin and axons by decreasing pro-inflammatory cytokines and impairing the migratory capacity of lymphocytes. Copyright © 2010 Elsevier B.V. All rights reserved.

  12. Allergic contact dermatitis to Plectranthus amboinicus masquerading as chronic leg ulcer.

    Science.gov (United States)

    Chang, Shyue-Luen; Chang, Ya-Ching; Yang, Chin-Hsun; Hong, Hong-Shang

    2005-12-01

    This report discusses a case of a 69-year-old woman who developed chronic non-healing leg ulcers after long-term topical use of Plectranthus amboinicus. The ulcer was proven to be allergic contact dermatitis to P. amboinicus by a patch test. The ulcer healed after discontinuation of P. amboinicus. To the best of our knowledge, this is the first reported case of allergic contact dermatitis to P. amboinicus masquerading as chronic leg ulcer.

  13. Subcutaneous immunoglobulin preserves muscle strength in chronic inflammatory demyelinating polyneuropathy

    DEFF Research Database (Denmark)

    Markvardsen, Lars Høj; Harbo, Thomas; Sindrup, Søren Hein

    2014-01-01

    evaluated after 3, 6 and 12 months. Primary end-points were changes in muscle strength evaluated by isokinetic dynamometry in four affected muscle groups and a composite score of muscle performance and function tests, including Medical Research Council (MRC) score, grip strength, 40-m walking test (40-MWT......BACKGROUND AND PURPOSE: Subcutaneous immunoglobulin (SCIG) is superior to placebo treatment for maintenance of muscle strength during 12 weeks in patients with chronic inflammatory demyelinating polyneuropathy (CIDP). The present study evaluated whether SCIG preserves muscle strength for 1 year......) and nine-hole peg test (9-HPT). Secondary end-points were changes of each of the listed parameters at each time point as well as an overall disability sum score (ODSS). RESULTS: The dose of SCIG was significantly unaltered during the follow-up period. Overall the isokinetic dynamometry value increased by 7...

  14. Ritual Continuity and “Failed Rituals” in a Winter Masquerade in the Italian Alps

    Directory of Open Access Journals (Sweden)

    Lia Zola

    2011-08-01

    Full Text Available Alpine winter masquerades, also known as carnival masquerades, may be regarded as one of the most complex phenomena within the field of study of cultural anthropology and folklore. In the Italian alpine chain some of them have been brought to a new life two decades ago after a long period of decline; since then, alpine winter masquerades have been the focus of a great interest by cultural anthropologists, theatre performers and other intellectuals. In some cases bringing a winter masquerade to new life has proved to be successful; in others, after a first, “reborn” performance, some celebrations abruptly stopped. My paper aims to explore issues of ritual continuity and ritual failure through a specific case-study, that of the carnival masquerade in Salbertrand, an alpine settlement of 485 inhabitants in the upper part of the Val di Susa, in the Italian western alpine chain.Les mascarades alpines d'hiver, aussi connues comme mascarades de carnaval, peuvent être considérées comme l’un des phénomènes les plus complexes dans le champ d'étude d'anthropologie culturelle et du folklore. Dans la chaîne alpine italienne, certains d'entre eux ont été amenés à une nouvelle vie il y a deux décennies, après une longue période de déclin. Dès lors, les mascarades alpines d'hiver ont été sujet d'intérêt majeur pour les anthropologues culturels, les acteurs de théâtre et d'autres intellectuels. Dans certains cas, réactiver une mascarade d'hiver s'est soldé par une réussite ; dans d'autres, après une première manifestation "renaissante" quelques célébrations se sont brusquement arrêtées. Mon article aspire à explorer les phénomènes de continuité et d'échec rituel à travres une étude de cas spécifique, celui de la mascarade de carnaval de Salbertrand, une localité alpine de 485 habitants dans la partie supérieure de Val De Suse, dans les Alpes occidentales italiennes.

  15. Phosphatidylcholine 36:1 concentration decreases along with demyelination in the cuprizone animal model and post-mortem of multiple sclerosis brain tissue.

    Science.gov (United States)

    Trépanier, Marc-Olivier; Hildebrand, Kayla D; Nyamoya, Stella D; Amor, Sandra; Bazinet, Richard P; Kipp, Markus

    2018-03-25

    Multiple sclerosis (MS) is a demyelinating and inflammatory disease. Myelin is enriched in lipids, and more specifically, oleic acid. The goal of this study was to evaluate the concentration of oleic acid following demyelination and remyelination in the cuprizone model, test if these changes occurred in specific lipid species, and whether differences in the cuprizone model correlate with changes observed in post-mortem human brains. Eight-week-old C57Bl/6 mice were fed a 0.2% cuprizone diet for 5 weeks and some animals allowed to recover for 11 days. Demyelination, inflammation, and lipid concentrations were measured in the corpus callosum. Standard fatty acid techniques and liquid chromatography combined with tandem mass spectrometry were performed to measure concentrations of fatty acids in total brain lipids and a panel of lipid species within the phosphatidylcholine (PC). Similar measurements were conducted in post-mortem brain tissues of MS patients and were compared to healthy controls. Five weeks of cuprizone administration resulted in demyelination followed by significant remyelination after 11 days of recovery. Compared to control, oleic acid was decreased after 5 weeks of cuprizone treatment and increased during the recovery phase. This decrease in oleic acid was associated with a specific decrease in the PC 36:1 pool. Similar results were observed in human post-mortem brains. Decreases in myelin content in the cuprizone model was accompanied with decreases in oleic acid concentration and is associated with PC 36:1 suggesting that specific lipids could be a potential biomarker for myelin degeneration. The biological relevance of oleic acid for disease progression remains to be verified. This article is protected by copyright. All rights reserved. This article is protected by copyright. All rights reserved.

  16. CT and MRI 'ring sign' may be due to demyelination: diagnostic pitfall.

    LENUS (Irish Health Repository)

    Kamel, M H

    2012-02-03

    We report a case of acute demyelinating encephalomyelitis (ADEM) in which both CT and MRI showed multiple ring-enhancing lesions suggestive of abscesses or brain tumour. This is a relatively rare phenomenon.

  17. A review of MRI evaluation of demyelination in cuprizone murine model

    Energy Technology Data Exchange (ETDEWEB)

    Krutenkova, E., E-mail: len--k@yandex.ru; Pan, E.; Khodanovich, M., E-mail: khodanovich@mail.tsu.ru [National Research Tomsk State University, Lenina pr., 36, Tomsk (Russian Federation)

    2015-11-17

    The cuprizone mouse model of non-autoimmune demyelination reproduces some phenomena of multiple sclerosis and is appropriate for validation and specification of a new method of non-invasive diagnostics. In the review new data which are collected using the new MRI method are compared with one or more conventional MRI tools. Also the paper reviewed the validation of MRI approaches using histological or immunohistochemical methods. Luxol fast blue histological staining and myelin basic protein immunostaining is widespread. To improve the accuracy of non-invasive conventional MRI, multimodal scanning could be applied. The new quantitative MRI method of fast mapping of the macromolecular proton fraction is a reliable biomarker of myelin in the brain and can be used for research of demyelination in animals. To date, a validation of MPF method on the CPZ mouse model of demyelination is not performed, although this method is probably the best way to evaluate demyelination using MRI.

  18. Demyelinating polyneuropathy, dermatomyositis, and interstitial pneumonitis associated with autoantibody against melanoma differentiation-associated gene 5

    Directory of Open Access Journals (Sweden)

    Yu-Hsiang Chiu

    2017-01-01

    Full Text Available Patients with serum anti-melanoma differentiation-associated gene 5 autoantibodies (anti-MDA5 are related to amyopathic dermatomyositis, especially in Asians. Here, we present a 46-year-old woman who was diagnosed with anti-MDA5-mediated demyelinating polyneuropathy clinically mimicking dermatomyositis. She had rapid progression of interstitial pneumonitis complicated with Pneumocystis jirovecii and Aspergillus pneumonia with septic shock. It is rare that patients with anti-MDA5-positive dermatomyositis present as demyelinating polyneuropathy.

  19. Inflammatory CNS demyelination:histopathologic correlation with in vivo quantitative proton MR spectroscopy

    OpenAIRE

    Bitsch, A; Bruhn, H; Vougioukas, V; Stringaris, AK; Lassmann, H; Frahm, J; Bruck, W

    1999-01-01

    BACKGROUND AND PURPOSE: The mechanisms behind the demyelination that is characteristic of multiple sclerosis (MS) are still poorly understood. The purpose of this study was to compare immunopathologic findings in demyelinating lesions of three patients with in vivo assessments obtained by quantitative proton MR spectroscopy (MRS).METHODS: Between four and seven stereotactic needle brain biopsies were performed in three young adults with diagnostically equivocal findings for MS. Axonal density...

  20. Alpha-lipoic acid mitigates toxic-induced demyelination in the corpus callosum by lessening of oxidative stress and stimulation of polydendrocytes proliferation.

    Science.gov (United States)

    Sanadgol, Nima; Golab, Fereshteh; Askari, Hassan; Moradi, Fatemeh; Ajdary, Marziyeh; Mehdizadeh, Mehdi

    2018-02-01

    Multiple Sclerosis (MS), is a disease that degenerates myelin in central nervous system (CNS). Reactive oxygen species (ROSs) are toxic metabolites, and accumulating data indicate that ROSs-mediated apoptosis of oligodendrocytes (OLGs) plays a major role in the pathogenesis of MS under oxidative stress conditions. In this study, we investigated the role of endogenous antioxidant alpha-lipoic acid (ALA) as ROSs scavenger in the OLGs loss and myelin degeneration during cuprizone (cup)-induced demyelination in the experimental model of MS. Our results have shown that ALA treatment significantly increased population of mature OLGs (MOG + cells), as well as decreased oxidative stress (ROSs, COX-2 and PGE2) and apoptosis mediators (caspase-3 and Bax/Bcl2 ratio) in corpus callosum (CC). Surprisingly, ALA significantly stimulates population of NG2 chondroitin sulfate proteoglycan positive glia (NG2 + cells or polydendrocytes), from week 4 afterward. Accordingly ALA could prevents apoptosis, delays demyelination and recruits OLGs survival and regeneration mechanisms in CC. We conclude that ALA has protective effects against toxic demyelination via reduction of redox signaling, and alleviation of polydendrocytes vulnerability to excitotoxic challenge.

  1. Experimental Demyelination and Axonal Loss Are Reduced in MicroRNA-146a Deficient Mice

    Directory of Open Access Journals (Sweden)

    Nellie A. Martin

    2018-03-01

    Full Text Available BackgroundThe cuprizone (CPZ model of multiple sclerosis (MS was used to identify microRNAs (miRNAs related to in vivo de- and remyelination. We further investigated the role of miR-146a in miR-146a-deficient (KO mice: this miRNA is differentially expressed in MS lesions and promotes differentiation of oligodendrocyte precursor cells (OPCs during remyelination, but its role has not been examined during demyelination.MethodsMicroRNAs were examined by Agilent Mouse miRNA Microarray in the corpus callosum during CPZ-induced demyelination and remyelination. Demyelination, axonal loss, changes in number of oligodendrocytes, OPCs, and macrophages/microglia was compared by histology/immunohistochemistry between KO and WT mice. Differential expression of target genes and proteins of miR-146a was analyzed in the transcriptome (4 × 44K Agilent Whole Mouse Genome Microarray and proteome (liquid chromatography tandem mass spectrometry of CPZ-induced de- and remyelination in WT mice. Levels of proinflammatory molecules in the corpus callosum were compared in WT versus KO mice by Meso Scale Discovery multiplex protein analysis.ResultsmiR-146a was increasingly upregulated during CPZ-induced de- and remyelination. The absence of miR-146a in KO mice protected against demyelination, axonal loss, body weight loss, and atrophy of thymus and spleen. The number of CNP+ oligodendrocytes was increased during demyelination in the miR-146a KO mice, while there was a trend of increased number of NG2+ OPCs in the WT mice. miR-146a target genes, SNAP25 and SMAD4, were downregulated in the proteome of demyelinating corpus callosum in WT mice. Higher levels of SNAP25 were measured by ELISA in the corpus callosum of miR-146a KO mice, but there was no difference between KO and WT mice during demyelination. Multiplex protein analysis of the corpus callosum lysate revealed upregulated TNF-RI, TNF-RII, and CCL2 in the WT mice in contrast to KO mice. The number of Mac3+ and

  2. Hepatosplenic Gamma/DeltaT-Cell Lymphoma Masquerading as Alcoholic Hepatitis and Methadone Withdrawal

    Directory of Open Access Journals (Sweden)

    H.A. Lopez Morra

    2007-09-01

    Full Text Available Hepatosplenic gamma/delta T-cell lymphoma is a rare neoplasm of mature gamma/delta T-cells with sinusoidal infiltration of spleen, liver, and bone marrow. Patients are predominantly adolescent and young adult males and usually present with marked hepatosplenomegaly. Pancytopenia is another common finding. Despite an initial response to treatment, patients have a median survival of one to two years. In this report, we document a case of alcoholic hepatitis and methadone withdrawal masquerading unsuspected, hepatosplenic gamma/delta T-cell lymphoma with unusual CD20 positivity.

  3. Jamar hand-held grip dynamometry in chronic inflammatory demyelinating polyneuropathy.

    Science.gov (United States)

    Rajabally, Yusuf A; Narasimhan, Manisha

    2013-02-15

    Few studies have evaluated the functional and electrophysiological correlates of hand-held grip dynamometry in neuromuscular disease. The practical value of normative values remains uncertain in clinical practice. We aimed to ascertain the value, clinical and electrophysiological correlates, as well as utility of normative values for grip dynamometry, in a cohort of subjects with chronic inflammatory demyelinating polyneuropathy (CIDP). We performed a cross-sectional study of 31 prospectively recruited subjects with CIDP, using Jamar hand-held grip dynamometry, conventional clinical assessments and electrophysiology. Grip dynamometry correlated highly with motor, sensory and functional scores in the upper limbs as well as with global function. There were significant correlations with summated median/ulnar, and ulnar compound muscle action potentials. Patients' readings were significantly lower than median normative values but comparable to fifth percentile normative figures. Jamar grip dynamometry is a reliable measure of global neurological status in patients with CIDP, not limited to upper limb or exclusively motor function. At optimum level of function, CIDP patients had comparable dynamometry recordings to fifth percentile normative values, which may represent a realistic aim for treated subjects with the disorder, with currently available therapies. Crown Copyright © 2012. Published by Elsevier B.V. All rights reserved.

  4. Bronchogenic cyst in the hepatogastric ligament masquerading as an esophageal mesenchymal tumor: a case report

    Science.gov (United States)

    Gou, Yawen; Wang, Yetao; Fang, Huaying; Xu, Xuemei; Yu, Wenyong; Zhang, Kaiguang; Yu, Yue

    2015-01-01

    Lesions occur in hepatogastric gap is common, but most of these lesions are from stomach, lower esophagus and pancreas lesions extending or transferring to the gap. Lesions occurred in the hepatogastric ligament are rare, especially bronchogenic cysts in the hepatogastric ligament. So far, there were no relevant cases reported. Here, we report a case of bronchogenic cyst in the hepatogastric ligament that masquerading as an esophageal mesenchymal tumor. A 24-year-old young man presented with abdominal bloating was diagnosed as esophageal mesenchymal tumor in previous hospital by gastroscopy, endoscopic ultrasonography (EUS) and computed tomography (CT) examination. For the sake of endoscopic submucosal dissection (ESD), the man was transferred to our hospital. During surgery, we found no lesions in lower esophagus, but external pressure ridge lesions in the distal esophagus right side wall. Then laparoscopic surgery and pathology confirmed as bronchogenic cyst in the hepatogastric ligament. We report what is, to the best of our knowledge, the first case of a bronchogenic cyst in the hepatogastric ligament masquerading as an esophageal mesenchymal tumor. PMID:26823885

  5. Anxiety, depression and fatigue at 5-year review following CNS demyelination.

    Science.gov (United States)

    Simpson, S; Tan, H; Otahal, P; Taylor, B; Ponsonby, A-L; Lucas, R M; Blizzard, L; Valery, P C; Lechner-Scott, J; Shaw, C; Williams, D; van der Mei, I

    2016-12-01

    Anxiety and depression are common in multiple sclerosis (MS). We evaluated the prevalence and factors associated with anxiety, depression and fatigue at the 5-year review of a longitudinal cohort study following a first clinical diagnosis of CNS demyelination (FCD). Cases with a FCD were recruited soon after diagnosis and followed annually thereafter. A variety of environmental, behavioural and clinical covariates were measured at five-year review. Anxiety and depression were measured using the Hospital Anxiety & Depression Scale (HADS), and fatigue by the Fatigue Severity Scale (FSS). Of the 236 cases, 40.2% had clinical anxiety (median HADS-A: 6.0), 16.0% had clinical depression (median HADS-D: 3.0), and 41.3% had clinical fatigue (median FSS: 4.56). The co-occurrence of all three symptoms was 3.76 times greater than expectation. Younger age, higher disability, concussion or other disease diagnosis were independently associated with a higher anxiety score; male sex, higher disability, being unemployed, less physical activity, and antidepressant and/or anxiolytic-sedative medication use were independently associated with a higher depression score. Higher disability, immunomodulatory medication use, other disease diagnosis and anxiolytic-sedative medication use were independently associated with having fatigue, while female sex, higher BMI, having had a concussion, being unemployed and higher disability were associated with a higher fatigue score. These results support previous findings of the commonality of anxiety, depression and fatigue in established MS and extend this to post-FCD and early MS cases. The clustering of the three symptoms indicates that they may share common antecedents. © 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  6. ′Wine Glass′ sign in recurrent postpartum hypernatremic osmotic cerebral demyelination

    Directory of Open Access Journals (Sweden)

    Aralikatte O Saroja

    2013-01-01

    Full Text Available Osmotic demyelination syndrome resulting from postpartum hypernatremia is a recently described entity wherein young women present with hypernatremic encephalopathy and white matter hyperintensities along with quadriparesis from rhabdomyolysis. It is an acute monophasic condition with acute hypernatremia occurring during puerperium with good recovery in majority of the patients with treatment. To the best of our knowledge, recurrent postpartum hypernatremia with encephalopathy, osmotic demyelination, and rhabdomyolysis has not been described. We present a young lady who had two episodes of reversible postpartum hypernatremic encephalopathy with rhabdomyolysis. Cerebral magnetic resonance imaging (MRI before treatment revealed osmotic demyelination on both occasions. During first admission MRI revealed hyperintensities in internal capsule and corpus callosum, and at second admission revealed more extensive white matter hyperintensity, which simulated the ′wine glass′ appearance.

  7. Neuronal loss, demyelination and volume change in the multiple sclerosis neocortex

    DEFF Research Database (Denmark)

    Carassiti, D; Altmann, D R; Petrova, N

    2017-01-01

    AIMS: Indices of brain volume [grey matter, white matter (WM), lesions] are being used as outcomes in clinical trials of patients with multiple sclerosis (MS). We investigated the relationship between cortical volume, the number of neocortical neurons estimated using stereology and demyelination....... Grey and WM demyelination was outlined on myelin basic protein immunostained sections, and expressed as percentages of cortex and WM respectively. RESULTS: In MS, the mean number of neurons was 14.9 ± 1.9 billion vs. 24.4 ± 2.4 billion in controls (P

  8. Paraneoplastic chronic demyelinating neuropathy and Lambert-Eaton myasthenic syndrome associated with multiple anti-neural antibodies and small-cell lung cancer.

    Science.gov (United States)

    Rozsa, Csilla; Vincent, Angela; Aranyi, Zsuzsanna; Kovacs, Gabor G; Komoly, Samuel; Illes, Zsolt

    2008-09-30

    Lambert-Eaton myasthenic syndrome (LEMS) developed in a patient with presumed chronic inflammatory demyelinating polyneuropathy (CIDP) and negative chest CT. Since antibodies against both Hu and voltage-gated calcium channel (VGCC) were detected, repeated chest CT was performed, which eventually showed a pulmonary mass lesion. Biopsy revealed small cell lung cancer (SCLC) indicating the importance of repeated chest CT in LEMS even when an existing autoimmune-like disease and negative CT may suggest an autoimmune origin. This is the first report of paraneoplastic CIDP and LEMS associated with anti-Hu, anti-VGCC and SCLC.

  9. Evidence of demyelination in mild cognitive impairment and dementia using a direct and specific magnetic resonance imaging measure of myelin content.

    Science.gov (United States)

    Bouhrara, Mustapha; Reiter, David A; Bergeron, Christopher M; Zukley, Linda M; Ferrucci, Luigi; Resnick, Susan M; Spencer, Richard G

    2018-04-18

    We investigated brain demyelination in aging, mild cognitive impairment (MCI), and dementia using magnetic resonance imaging of myelin. Brains of young and old controls and old subjects with MCI, Alzheimer's disease, or vascular dementia were scanned using our recently developed myelin water fraction (MWF) mapping technique, which provides greatly improved accuracy over previous comparable methods. Maps of MWF, a direct and specific myelin measure, and relaxation times and magnetization transfer ratio, indirect and nonspecific measures, were constructed. MCI subjects showed decreased MWF compared with old controls. Demyelination was greater in Alzheimer's disease or vascular dementia. As expected, decreased MWF was accompanied by decreased magnetization transfer ratio and increased relaxation times. The young subjects showed greater myelin content than the old subjects. We believe this to be the first demonstration of myelin loss in MCI, Alzheimer's disease, and vascular dementia using a method that provides a quantitative magnetic resonance imaging-based measure of myelin. Our findings add to the emerging evidence that myelination may represent an important biomarker for the pathology of MCI and dementia. This study supports the investigation of the role of myelination in MCI and dementia through use of this quantitative magnetic resonance imaging approach in clinical studies of disease progression, relationship of functional status to myelination status, and therapeutics. Furthermore, mapping MWF may permit myelin to serve as a therapeutic target in clinical trials. Copyright © 2018. Published by Elsevier Inc.

  10. A case of reactive arthritis: a great masquerader.

    Science.gov (United States)

    Ngaruiya, Christine M; Martin, Ian B K

    2013-01-01

    Reactive arthritis is an inflammatory condition with multiorgan system disease potential. Because the standard constellation of symptoms in Reiter syndrome (arthritis, conjunctivitis, and urethritis) is not typically present in all patients, the disease can be easily overlooked if clinical suspicion is not high upon presentation. To highlight the importance of recognizing the potential of this disease in patients with a history of either gastrointestinal or genitourinary illnesses, we present the case of a young healthy male presented on multiple occasions later diagnosed with Reiter syndrome in the setting of a recent diagnosis of prostatitis. He was noted to have classical symptoms including conjunctivitis and arthritis. He was treated with nonsteroidal anti-inflammatory drugs during a brief hospital stay and did well. Although reactive arthritis is an easily managed disease, it is easily missed particularly in young otherwise healthy patients who may not present with classic symptoms. Vigilance with regard to patients with vague seemingly unrelated complaints particularly with a history of gastrointestinal- or genitourinary-related illnesses deserves consideration for this disease process.

  11. Mucormycosis in a surgical defect masquerading as osteomyelitis: a ...

    African Journals Online (AJOL)

    This fatal fungal disease needs a prompt and early definitive diagnosis, aggressive surgical therapy and high dose anti-fungal therapy. Here, we present a case report of Mucormycosis in a 64 year elderly diabetic male patient who was previously operated for myiasis and also the extensive review of the literature of the ...

  12. Tubercular biliary stricture – a malignant masquerade | Vijay | South ...

    African Journals Online (AJOL)

    Hilar cholangiocarcinoma is the most common cause of a stricture in the hilar region, and hilar stricture in the absence of any previous surgical intervention should be considered to indicate malignant disease until proven otherwise. We present a rare case of isolated hilar tubercular stricture, all the features of which were ...

  13. Retained soft contact lens masquerading as a chalazion: a case report.

    Science.gov (United States)

    Agarwal, Pankaj Kumar; Ahmed, Taha Y; Diaper, Charles J M

    2013-02-01

    A misplaced contact lens is a common ocular emergency presenting to the eye casualty. We report a case of lost soft contact lens which migrated in the lid and presented 13 years later with symptomatic eye lid swelling. Authors in the past have reported migration and subsequent retention of lost hard lenses in locations such as the superior fornix and eyelid. To the best of our knowledge, misplaced soft contact lens masquerading as a chalazion has not been reported in the literature. Consideration should be given to the possibility of a retained contact lens in a patient with a history of a lost or misplaced lens, and examination of the ocular surface with double eversion of the upper lid should be performed.

  14. Retained soft contact lens masquerading as a chalazion: A case report

    Directory of Open Access Journals (Sweden)

    Pankaj Kumar Agarwal

    2013-01-01

    Full Text Available A misplaced contact lens is a common ocular emergency presenting to the eye casualty. We report a case of lost soft contact lens which migrated in the lid and presented 13 years later with symptomatic eye lid swelling. Authors in the past have reported migration and subsequent retention of lost hard lenses in locations such as the superior fornix and eyelid. To the best of our knowledge, misplaced soft contact lens masquerading as a chalazion has not been reported in the literature. Consideration should be given to the possibility of a retained contact lens in a patient with a history of a lost or misplaced lens, and examination of the ocular surface with double eversion of the upper lid should be performed.

  15. Squamous Cell Carcinoma Arising in Mature Teratoma of the Ovary Masquerading as Abdominal Tuberculosis

    Directory of Open Access Journals (Sweden)

    Shreeya Taresh Indulkar

    2018-01-01

    Full Text Available Pure squamous cell carcinoma (SCC of the ovary is rare. SCC can arise in a mature teratoma (MT, ovarian endometriosis or in a Brenner tumor. SCC is the most common malignant transformation arising in MT and comprises 80% of all cases. Such neoplastic transformations are extremely difficult either to predict or detect early. The mechanism of malignant transformation has not been completely understood. Due to the rarity and the aggressive course, diagnosis and treatment constitute a big challenge. We report a case of SCC arising in MT presenting with a huge abdominopelvic mass and abundant peritoneal collections clinically masquerading as abdominal tuberculosis. A review of literature with special emphasis on prognosis and treatment modalities is also presented.

  16. Granulocytic sarcoma masquerading as Ewing′s sarcoma: A diagnostic dilemma

    Directory of Open Access Journals (Sweden)

    Haresh Kunhi

    2008-01-01

    Full Text Available An eleven-year-old boy presented with a swelling in his left elbow. Radiologically the features were that of an Ewing′s sarcoma involving the ulna. Histopathology showed small round cell tumor strongly positive for Monoclonal Imperial Cancer research fund 2 (MIC2 antigen. Similar cells in the bone marrow were involved with MIC2 positivity. The patient developed skin lesions, which on biopsy were found to be chloromas. The initial biopsies were reevaluated with special stains revealing granulocytic sarcomas in acute myeloid leukemia masquerading as Ewing′s due to its MIC2 positivity. The possibility of myeloid neoplasms should be considered routinely with known MIC2 positive round cell tumors.

  17. A Case of Hyperemesis Gravidarum due to Gastric Cancer Masquerading as Preeclampsia

    Directory of Open Access Journals (Sweden)

    Daniel R. Hersh

    2011-12-01

    Full Text Available Nausea and vomiting are symptoms frequently seen in normal pregnancy. We report a patient with gastric carcinoma who presented with severe hyperemesis gravidarum that led to extreme volume depletion, hypertension, proteinuria, and acute renal failure. A 35-year-old woman (para 2-1-0-1 with a prenatal course significant for persistent nausea, vomiting, and poor weight gain presented at 36 weeks' gestation with elevated blood pressure (157/114 mm Hg, proteinuria (4+, hypochloremic metabolic alkalosis, and severe intravascular volume contraction. A presumptive diagnosis of severe preeclampsia was made, the patient was given intravenous MgSO4, and cesarean delivery was accomplished uneventfully. When significant emesis persisted in the postoperative period, esophagogastroduodenoscopy revealed an antral/prepyloric mass with a biopsy-proven poorly differentiated adenocarcinoma. To our knowledge, this is the first report of a case of hyperemesis gravidarum with gastric cancer masquerading as preeclampsia.

  18. Fashion pictures and women pictures in French women magazine. Normatives parades or strategic masquerade?

    Directory of Open Access Journals (Sweden)

    Justine MARILLONNET

    2014-12-01

    Full Text Available This article proposes to study the fashion pictures within a representative sample of French women magazines. Based on the method of “sémiologie des indices”, used to analyze a corpus of woman’s body and fashion staged, this paper addresses the issue of new functions granted to the stereotyping process. Starting to the assumption of a contract between audience and media, this analysis shows that the gender stereotype may be prescribed, up to a caricature of the womanhood, to the female readers identified as co-producers of the media discourse. Demonstrating both the co-existence of a plurality of gender representations in its pictures and their variability, this work proposes the concept of strategic masquerade to apprehend the possible new functions for the process of gender stereotyping in media.

  19. Perirectal Abscess Masquerading as Cauda Equina Syndrome in an Otherwise Healthy 12-Year-Old Child

    Directory of Open Access Journals (Sweden)

    Dylan Dean

    2014-01-01

    Full Text Available A 12-year-old boy was brought to an urgent care center for fever, back pain, and abnormal gait. In addition to back pain, the patient was found to be persistently febrile but also had decreased perianal sensation and bowel incontinence. He was therefore referred to the emergency department where his back pain improved without medication but he was still febrile with bowel incontinence and persistently decreased perianal sensation. An MRI was ordered to evaluate possible cauda equina syndrome and revealed a perirectal abscess. The child ultimately underwent an exam under anesthesia with pediatric surgery and had a drain placed. This case highlights a unique presentation of perirectal abscess masquerading as cauda equina syndrome. A discussion of important considerations in emergency room diagnosis and management is presented.

  20. A masquerade is not watched from one spot: reassessing the study of African religions

    Directory of Open Access Journals (Sweden)

    Umar Habila Dadem Danfulani

    1999-01-01

    Full Text Available The search for an adequate methodological approach to the study of African Religions in their multiformity has been a fervent one. In this paper, approaches to the study of African Religions today are proposed. These are polymethodic and multidimentional approaches, the contextual study of African Religions, the historical approaches (demonstrated in the art of masquerading, the need to balance synchronic and diachronic approaches, the use of art and iconography in the study and female studies. The fifth focuses on studying African Religions as insider. The author also examines the problems of the "insider" and the politics of doing research in history of religions in a Nigerian university.The conclusion strongly recommends a multidisciplinary approach for studying African Religions.

  1. Lesser-known myelin-related disorders: focal tumour-like demyelinating lesions.

    Science.gov (United States)

    Jiménez Arango, J A; Uribe Uribe, C S; Toro González, G

    2015-03-01

    Focal tumour-like demyelinating lesions are defined as solitary demyelinating lesions with a diameter greater than 2 cm. In imaging studies, these lesions may mimic a neoplasm or brain abscess; as a result, invasive diagnostic and therapeutic measures may be performed that will in some cases increase morbidity. Our aim was to analyse and characterise these lesions according to their clinical, radiological, and pathological characteristics, and this data in addition to our literature review will contribute to a better understanding of these lesions. This descriptive study includes 5 cases with pathological diagnoses. We provide subject characteristics gathered through reviewing their clinical, radiology, and pathology reports. Patients' ages ranged from 12 to 60 years; 3 patients were female. The time delay between symptom onset and hospital admission was 3 to 120 days. Clinical manifestations were diverse and dependent on the location of the lesion, pyramidal signs were found in 80% of patients, there were no clinical or radiological signs of spinal cord involvement, and follow-up times ranged from 1 to 15 years. Brain biopsy is the gold standard for the diagnosis of demyelinating tumour-like lesions; however, their clinical features, along with several magnetic resonance imaging features such as open ring enhancement, venular enhancement, the presence of glutamate in spectroscopy, and others, may be sufficient to differentiate neoplastic lesions from focal tumour-like demyelinating lesions. Copyright © 2012 Sociedad Española de Neurología. Published by Elsevier Espana. All rights reserved.

  2. Delayed-onset adrenoleukodystrophy after cerebral contusion : progressive pattern of demyelination on serial MR imaging

    Energy Technology Data Exchange (ETDEWEB)

    Yoon, Kwon Ha; Suh, Dae chul; Lee, Sang Ahm; Lee, Ho Kyu; Choi, Choong Gon; Yoo, Shi Joon [Ulsan Univ. College of Medicine, Seoul (Korea, Republic of)

    1996-08-01

    We described serial MR findings in a 20-year-old male with adrenoleuko-dystrophy who presented progressive neurologic deterioration after cerebral contusion. On MR imaging, progressive demyelination was predominant in the white matter of the right temporal lobe as well as tn the parietalobe at the site of prior trauma and exteded into the contralateral hemisphere through the anterior commissure.

  3. Mitochondrial DNA double-strand breaks in oligodendrocytes cause demyelination, axonal injury, and CNS inflammation

    DEFF Research Database (Denmark)

    Madsen, Pernille M.; Pinto, Milena; Patel, Shreyans

    2017-01-01

    (PLP:mtPstI mice) by expressing a mitochondrial-targeted endonuclease, mtPstI, starting at 3 weeks of age. In both female and male mice, DSBs of oligodendroglial mtDNA caused impairment of locomotor function, chronic demyelination, glial activation, and axonal degeneration, which became more severe...

  4. Anti-beta-tubulin antibodies have no diagnostic value in patients with chronic inflammatory demyelinating polyneuropathy

    NARCIS (Netherlands)

    van Schaik, I. N.; Vermeulen, M.; van Doorn, P. A.; Brand, A.

    1995-01-01

    High-titre anti-beta-tubulin antibodies were recently reported to occur in over 50% of sera from patients with chronic inflammatory demyelinating polyneuropathy (CIDP). It was concluded that these antibodies may help to distinguish CIDP from other neuropathies and that they are diagnostically

  5. Supramaximal Stimulus Intensity as a Diagnostic Tool in Chronic Demyelinating Neuropathy

    Directory of Open Access Journals (Sweden)

    Vivien Parker

    2016-01-01

    Full Text Available Objective. The ability to correctly identify chronic demyelinating neuropathy can have important therapeutic and prognostic significance. The stimulus intensity value required to obtain a supramaximal compound muscle action potential amplitude is a commonly acquired data point that has not been formally assessed as a diagnostic tool in routine nerve conduction studies to identify chronic neuropathies. We postulated that this value was significantly elevated in chronic demyelinating neuropathy. Methods. We retrospectively reviewed electrophysiology laboratory records to compare the stimulus intensity values recorded during median and ulnar motor nerve conduction studies. The groups studied included normal controls (n=42 and the following diagnostic categories: chronic inflammatory demyelinating neuropathy (CIDP (n=20, acquired inflammatory demyelinating neuropathy (AIDP (n=13, Charcot Marie Tooth (CMT type 1 or 4C (n=15, carpal tunnel syndrome (CTS (n=11, and amyotrophic lateral sclerosis (ALS (n=18. Results. Supramaximal intensities were significantly higher in patients with CMT (median nerve: 43.4 mA and CIDP (median nerve: 38.9 mA, whereas values similar to normal controls (median nerve: 25.3 mA were obtained in ALS, CTS, and AIDP. Conclusions. Supramaximal stimulus intensity may be used as an additional criterion to identify the pathophysiology of neuropathy. We postulate that endoneurial hypertrophic changes may increase electrical impedance and thus the threshold of excitation at nodes of Ranvier.

  6. Demyelination in Multiple Sclerosis: Reprogramming Energy Metabolism and Potential PPARγ Agonist Treatment Approaches.

    Science.gov (United States)

    Vallée, Alexandre; Lecarpentier, Yves; Guillevin, Rémy; Vallée, Jean-Noël

    2018-04-16

    Demyelination in multiple sclerosis (MS) cells is the site of several energy metabolic abnormalities driven by dysregulation between the opposed interplay of peroxisome proliferator-activated receptor γ (PPARγ) and WNT/β-catenin pathways. We focus our review on the opposing interactions observed in demyelinating processes in MS between the canonical WNT/β-catenin pathway and PPARγ and their reprogramming energy metabolism implications. Demyelination in MS is associated with chronic inflammation, which is itself associated with the release of cytokines by CD4⁺ Th17 cells, and downregulation of PPARγ expression leading to the upregulation of the WNT/β-catenin pathway. Upregulation of WNT/β-catenin signaling induces activation of glycolytic enzymes that modify their energy metabolic behavior. Then, in MS cells, a large portion of cytosolic pyruvate is converted into lactate. This phenomenon is called the Warburg effect, despite the availability of oxygen. The Warburg effect is the shift of an energy transfer production from mitochondrial oxidative phosphorylation to aerobic glycolysis. Lactate production is correlated with increased WNT/β-catenin signaling and demyelinating processes by inducing dysfunction of CD4⁺ T cells leading to axonal and neuronal damage. In MS, downregulation of PPARγ decreases insulin sensitivity and increases neuroinflammation. PPARγ agonists inhibit Th17 differentiation in CD4⁺ T cells and then diminish release of cytokines. In MS, abnormalities in the regulation of circadian rhythms stimulate the WNT pathway to initiate the demyelination process. Moreover, PPARγ contributes to the regulation of some key circadian genes. Thus, PPARγ agonists interfere with reprogramming energy metabolism by directly inhibiting the WNT/β-catenin pathway and circadian rhythms and could appear as promising treatments in MS due to these interactions.

  7. Deep gray matter demyelination detected by magnetization transfer ratio in the cuprizone model.

    Directory of Open Access Journals (Sweden)

    Sveinung Fjær

    Full Text Available In multiple sclerosis (MS, the correlation between lesion load on conventional magnetic resonance imaging (MRI and clinical disability is weak. This clinico-radiological paradox might partly be due to the low sensitivity of conventional MRI to detect gray matter demyelination. Magnetization transfer ratio (MTR has previously been shown to detect white matter demyelination in mice. In this study, we investigated whether MTR can detect gray matter demyelination in cuprizone exposed mice. A total of 54 female C57BL/6 mice were split into one control group ( and eight cuprizone exposed groups ([Formula: see text]. The mice were exposed to [Formula: see text] (w/w cuprizone for up to six weeks. MTR images were obtained at a 7 Tesla Bruker MR-scanner before cuprizone exposure, weekly for six weeks during cuprizone exposure, and once two weeks after termination of cuprizone exposure. Immunohistochemistry staining for myelin (anti-Proteolopid Protein and oligodendrocytes (anti-Neurite Outgrowth Inhibitor Protein A was obtained after each weekly scanning. Rates of MTR change and correlations between MTR values and histological findings were calculated in five brain regions. In the corpus callosum and the deep gray matter a significant rate of MTR value decrease was found, [Formula: see text] per week ([Formula: see text] and [Formula: see text] per week ([Formula: see text] respectively. The MTR values correlated to myelin loss as evaluated by immunohistochemistry (Corpus callosum: [Formula: see text]. Deep gray matter: [Formula: see text], but did not correlate to oligodendrocyte density. Significant results were not found in the cerebellum, the olfactory bulb or the cerebral cortex. This study shows that MTR can be used to detect demyelination in the deep gray matter, which is of particular interest for imaging of patients with MS, as deep gray matter demyelination is common in MS, and is not easily detected on conventional clinical MRI.

  8. Disseminated tuberculosis masquerading as a presentation of systemic lupus erythematosus.

    Science.gov (United States)

    Li, Justin C-H; Fong, Warren; Wijaya, Limin; Leung, Ying Y

    2018-01-01

    Tuberculosis (TB) infection is the endemic in Asia-Pacific region. Miliary TB is a disseminated form which may present similarly as autoimmune conditions. Here we describe a 17-year-old girl who had miliary TB with manifestations mimicking new-onset systemic lupus erythematosus (SLE) including oral ulcers, serositis, cytopenia, proteinuria and raised autoantibody titers. Complex associations between SLE and TB are highlighted. High index of clinical suspicion for TB infection is needed upon presentations resembling immune diseases like SLE. © 2017 Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd.

  9. Acute Sickle Cell Orbitopathy Masquerading as Orbital Cellulitis.

    Science.gov (United States)

    Stewart, Christopher M; Sipkova, Zuzana; Hildebrand, Goran D; Norris, Jonathan H

    2018-01-01

    Sickle cell orbitopathy is a rare manifestation of a sickle cell crisis and can closely mimic orbital cellulitis, both clinically and radiologically. We present a case where a child presented with what was thought to be orbital cellulitis and was treated accordingly. Subtle radiologic features of sickle cell orbitopathy were initially overlooked, resulting in an incorrect diagnosis and a treatment delay for the patient. Correctly treated most cases resolve with no adverse affects, highlighting how important it is to consider acute sickle cell orbitopathy in the differential diagnosis of acute periorbital swelling in children with sickle disease.

  10. Glia Disease and Repair-Remyelination

    DEFF Research Database (Denmark)

    Franklin, Robin J M; Goldman, Steven A

    2015-01-01

    The inability of the mammalian central nervous system (CNS) to undergo spontaneous regeneration has long been regarded as a central tenet of neurobiology. However, although this is largely true of the neuronal elements of the adult mammalian CNS, save for discrete populations of granular neurons...... it fails and the consequences of its failure; and discuss approaches for therapeutically enhancing remyelination in demyelinating diseases of both children and adults, both by stimulating endogenous oligodendrocyte progenitor cells and by transplanting these cells into demyelinated brain....

  11. A case of congenital myopathy masquerading as paroxysmal dyskinesia

    Directory of Open Access Journals (Sweden)

    Harsh Patel

    2014-01-01

    Full Text Available Gastroesophageal reflux (GER disease is a significant comorbidity of neuromuscular disorders. It may present as paroxysmal dyskinesia, an entity known as Sandifer syndrome. A 6-week-old neonate presented with very frequent paroxysms of generalized stiffening and opisthotonic posture since day 22 of life. These were initially diagnosed as seizures and he was started on multiple antiepileptics which did not show any response. After a normal video electroencephalogram (VEEG was documented, possibility of dyskinesia was kept. However, when he did not respond to symptomatic therapy, Sandifer syndrome was thought of and GER scan was done, which revealed severe GER. After his symptoms got reduced to some extent, a detailed clinical examination revealed abnormal facies with flaccid quadriparesis. Muscle biopsy confirmed the diagnosis of a specific congenital myopathy. On antireflux measures, those episodic paroxysms reduced to some extent. Partial response to therapy in GER should prompt search for an underlying secondary etiology.

  12. Patient with neuromyelitis optica and inflammatory demyelinating lesions comprising whole spinal cord from C2 level till conus: case report

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    Pavlisa Goran

    2009-10-01

    Full Text Available Abstract Background Neuromyelitis optica (NMO is an idiopathic, severe, inflammatory demyelinating disease of the central nervous system, that causes severe optic neuritis and myelitis attacks. Early discrimination between multiple sclerosis (MS and NMO is important, as optimum treatment for both diseases may differ considerably. Case Presentation We report a case of a patient who initially presented as longitudinally extensive transverse myelitis (LETM, having spastic upper extremities diparesis and spastic paraplegia, C2/C3 sensory level and urinary incontinence, as well as extensive inflammatory spinal cord lesions from C2 level to conus. After 5 months the patient had another attack of transverse myelitis, had electrophysiological findings consistent with optic neuritis, was seropositive for NMO-IgG (aquaporin-4 IgG and thus fulfilled NMO diagnostic criteria. Following treatment of disease attacks with pulse corticosteroid therapy and intravenous immunoglobulins, we included oral azathioprine in a combination with oral prednisone in the therapy. Since there was no significant clinical improvement, we decided to use cyclophosphamide therapy, which resulted in good clinical improvement and gradual decrease of cord swelling. Conclusion In this NMO case report we wanted to emphasize the extensiveness of inflammatory spinal cord changes in our patient, from C2 level to conus. In the conclusion it is important to say that accurate, early diagnosis and distinction from MS is critical to facilitate initiation of immunosuppressive therapy for attack prevention.

  13. Sellar Wegener Granulomatosis Masquerading as Cabergoline-Resistant Prolactinoma.

    Science.gov (United States)

    Eli, Ilyas M; Raheja, Amol; Corn, Heather J; Simmons, Debra L; Palmer, Cheryl Ann; Couldwell, William T

    2016-11-01

    Pituitary manifestation of Wegener granulomatosis (WG) is extremely rare. When there is pituitary involvement, the granulomatous inflammatory lesions involving the pituitary gland may appear several months to years after the primary diagnosis. We present a case of a 32-year-old woman who presented with galactorrhea, amenorrhea, and elevated serum prolactin levels. Imaging demonstrated a sellar lesion with characteristics of a pituitary macroadenoma. Treatment with cabergoline was initiated, but the tumor continued to grow during a 6-month period. Subsequent surgical exploration revealed a chronic inflammatory lesion; the patient subsequently was diagnosed with WG based on laboratory evaluation and further systemic manifestations. She had a favorable clinical and radiologic response with immunosuppressive doses of glucocorticoids and rituximab. This case appears to be the first reported of a patient with unknown WG in whom the diagnosis was established after she presented with a sellar lesion mimicking a prolactin-secreting pituitary adenoma on initial presentation requiring surgical resection. The only endocrine abnormality discovered was moderate hyperprolactinemia. Sellar lesions with only moderate elevations in serum prolactin, particularly those that are refractory to medical management with a dopamine agonist, should prompt further investigation to confirm the diagnosis. WG should be part of the differential diagnosis of inflammatory lesions in the sella, the identification of which can facilitate early diagnosis and treatment of this systemic disease for optimal outcome. Copyright © 2016 Elsevier Inc. All rights reserved.

  14. Liposarcoma of the Spermatic Cord Masquerading as an Inguinal Hernia

    Directory of Open Access Journals (Sweden)

    William Londeree

    2014-01-01

    Full Text Available This is a case of a 70-year-old male who presented with a mass in his right testicle. He was treated with antibiotics for epididymitis while undergoing serial ultrasounds for one year due to testicular swelling and pain. His fourth ultrasound revealed a mild hydrocele with a large paratesticular mass of undescribed size, superior to the right testicle, thought to be an inguinal hernia. Preoperative CT scan demonstrated a large fat-containing inguinal hernia extending into the scrotal sac. An inguinal hernia repair was complicated by fatty tissue surrounding the testicle requiring a right orchiectomy. Pathology review of the tissue demonstrated well-differentiated liposarcoma with a small focus of dedifferentiation grade 2 tumor. Tumor was identified at the inked margins indicating an incomplete resection. It was decided that no further surgical intervention was needed and the patient would undergo surveillance for local tumor recurrence. Six-month follow-up MRI scan was negative for any recurrence of disease. A liposarcoma presenting as a paratesticular mass with spermatic cord involvement is rare, and imaging studies may fail to distinguish a liposarcoma from normal adipose tissue.

  15. Hepatic Angiosarcoma Masquerading as Hemangioma: A Challenging Differential Diagnosis

    Directory of Open Access Journals (Sweden)

    Ana Rita Garcia

    2017-10-01

    Full Text Available Hemangiomas are usually diagnosed based on ultrasound findings. The presence of symptoms, rapid growth or atipical imagiological findings should make us consider other diagnoses, including malignant tumors such as angiosarcomas. We describe the case of a previously healthy 46-year-old female without a history of exposure to carcinogens who presented with abdominal pain for two months. Diagnostic work-up revealed elevated gamma-glutamyl transferase and lactate dehydrogenase levels. Abdominal ultrasound described a large nodular lesion in the right lobe of the liver described as a hemangioma. One month later, a computed tomography-scan was made and revealed the same lesion, which had grown from 13.5 to 20 cm, maintaining typical imaging characteristics of a hemangioma. A right hepatectomy was performed and pathology revealed an angiosarcoma. After surgery, a positron emission tomography-computed tomography scan showed hepatic and bone metastasis. The patient started taxane-based chemotherapy and lumbar palliative radiotherapy, but died 10 months after surgery. This case shows how difficult it is to diagnose hepatic angiosarcoma relying only on imaging findings. Two abdominal computed tomography -scans were performed and none suggested this diagnosis. Angiosarcoma is a very aggressive tumour with an adverse prognosis. Surgery is the only curative treatment available. However, it is rarely feasible due to unresectable disease or distant metastasis.

  16. Massively dilated right atrium masquerading as a mediastinal tumor

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    Thomas Schroeter

    2011-04-01

    Full Text Available Severe tricuspid valve insufficiency causes right atrial dilatation, venous congestion, and reduced atrial contractility, and may eventually lead to right heart failure. We report a case of a patient with severe tricuspid valve insufficiency, right heart failure, and a massively dilated right atrium. The enormously dilated atrium compressed the right lung, resulting in a radiographic appearance of a mediastinal tumor. Tricuspid valve repair and reduction of the right atrium was performed. Follow up examination revealed improvement of liver function, reduced peripheral edema and improved New York Heart Association (NYHA class. The reduction of the atrial size and repair of the tricuspid valve resulted in a restoration of the conduit and reservoir function of the right atrium. Given the chronicity of the disease process and the long-standing atrial fibrillation, there is no impact of this operation on right atrial contraction. In combination with the reconstruction of the tricuspid valve, the reduction atrioplasty will reduce the risk of thrombembolic events and preserve the right ventricular function.

  17. Application of the 2012 revised diagnostic definitions for paediatric multiple sclerosis and immune-mediated central nervous system demyelination disorders

    NARCIS (Netherlands)

    van Pelt, E. Danielle; Neuteboom, Rinze F.; Ketelslegers, Immy A.; Boon, Maartje; Catsman-Berrevoets, Coriene E.; Hintzen, Rogier Q.

    Background Recently, the International Paediatric Multiple Sclerosis Study Group (IPMSSG) definitions for the diagnosis of immune-mediated acquired demyelinating syndromes (ADS) of the central nervous system, including paediatric multiple sclerosis (MS), have been revised. Objective To evaluate the

  18. Incidence and Natural History of Idiopathic Chronic Inflammatory Demyelinating Polyneuropathy: A Population-Based Study in Iceland.

    Science.gov (United States)

    Hafsteinsdottir, Brynhildur; Olafsson, Elias

    2016-01-01

    We report a population-based study conducted in Iceland to determine the incidence, clinical characteristics and prognosis of idiopathic chronic inflammatory demyelinating polyneuropathy (CIDP) during a 21-year period. Cases were identified from the records of all practicing neurologists in the country, the only neurology department in the country and both neurophysiology laboratories. All index cases met the 2010 European Federation of Neurological Societies/Peripheral Nerve Society criteria for CIDP. Nineteen individuals fulfilled the diagnostic criteria during the study period. The average annual incidence was 0.3/100,000 (95% CI 0.04-2.47). There were 14 men (74%) in a gender ratio of 1:2.8. The mean age at diagnosis was 57 (range 19-81 years): women, 36 years and men, 63 years; p = 0.0006. The disease course was remitting-relapsing in 21% and chronic progressive or monophasic in 79%. The average length of follow-up was 6.9 years. The standardized mortality ratio for the 21-year study period was 0.9 (95% CI 0.3-2.2). We believe we have identified all diagnosed with CIDP in Iceland during a 21-year period. Many had no or only limited disease progression over the years and mortality is not increased compared with the general population. © 2016 S. Karger AG, Basel.

  19. Inhibition of GABA A receptor improved special memory impairment in the local model of demyelination in rat hippocampus.

    Science.gov (United States)

    Mousavi Majd, Alireza; Ebrahim Tabar, Forough; Afghani, Arghavan; Ashrafpour, Sahand; Dehghan, Samaneh; Gol, Mohammad; Ashrafpour, Manouchehr; Pourabdolhossein, Fereshteh

    2018-01-15

    Cognitive impairment and memory deficit are common features in multiple Sclerosis patients. The mechanism of memory impairment in MS is unknown, but neuroimaging studies suggest that hippocampal demyelination is involved. Here, we investigate the role of GABA A receptor on spatial memory in the local model of hippocampal demyelination. Demyelination was induced in male Wistar rats by bilaterally injection of lysophosphatidylcholine (LPC) 1% into the CA1 region of the hippocampus. The treatment groups were received daily intraventricular injection of bicuculline (0.025, 0.05μg/2μl/animal) or muscimol (0.1, 0.2μg/2μl/animal) 5days after LPC injection. Morris Water Maze was used to evaluate learning and memory in rats. We used Luxol fast blue staining and qPCR to assess demyelination extention and MBP expression level respectively. Immunohistochemistry (IHC) for CD45 and H&E staining were performed to assess inflammatory cells infiltration. Behavioral study revealed that LPC injection in the hippocampus impaired learning and memory function. Animals treated with both doses of bicuculline improved spatial learning and memory function; however, muscimol treatment had no effect. Histological and MBP expression studies confirmed that demylination in LPC group was maximal. Bicuculline treatment significantly reduced demyelination extension and increased the level of MBP expression. H&E and IHC results showed that bicuculline reduced inflammatory cell infiltration in the lesion site. Bicuculline improved learning and memory and decreased demyelination extention in the LPC-induced hippocampal demyelination model. We conclude that disruption of GABAergic homeostasis in hippocampal demyelination context may be involved in memory impairment with the implications for both pathophysiology and therapy. Copyright © 2017. Published by Elsevier B.V.

  20. Acute inflammatory demyelinating polyneuropathy associated with pegylated interferon α 2a therapy for chronic hepatitis C virus infection

    OpenAIRE

    Khiani, Vijay; Kelly, Thomas; Shibli, Adeel; Jensen, Donald; Mohanty, Smruti R

    2008-01-01

    The combination of pegylated interferon (Peg-IFN) and ribavirin is the standard of care for chronic hepatitis C virus (HCV) infection treatment. In general, common side effects related to this combination therapy are mild and are very well tolerated. However, peripheral neuropathy including demyelinating polyneuropathy related to Peg-IFN is extremely rare. We present the first case of an acute inflammatory demyelinating polyneuropathy (AIDP) associated with Peg-IFN-α 2a (Pegasys) after 16 wk ...

  1. Overview of the pathogenesis and treatment of chronic inflammatory demyelinating polyneuropathy with intravenous immunoglobulins

    Directory of Open Access Journals (Sweden)

    Mohamed Mahdi-Rogers

    2010-03-01

    Full Text Available Mohamed Mahdi-Rogers, Yusuf A RajaballyNeuromuscular Clinic, Department of Neurology, University Hospitals of Leicester, Leicester, UKAbstract: Chronic inflammatory demyelinating polyneuropathy (CIDP is an acquired heterogeneous disorder of immune origin affecting the peripheral nerves, causing motor weakness and sensory symptoms and signs. The precise pathophysiology of CIDP remains uncertain although B and T cell mechanisms are believed to be implicated. Intravenous immunoglobulins (IVIg have been shown in a number of trials to be an effective treatment for CIDP. IVIg is thought to exert its immunomodulatory effects by affecting several components of the immune system including B-cells, T-cells, macrophages and complement. This article provides an overview of the pathogenesis of CIDP and of its treatment with IVIg.Keywords: chronic inflammatory demyelinating polyneuropathy, intravenous immunoglobulin, pathogenesis, treatment

  2. Osmotic demyelination syndrome with recent chemotherapy in normonatremic patient: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Sungjae; Baek, Hye Jin; Jung, Hyun Kyung; Kim, Seon Jeong; Lee, Yedaun; Lee, Kwaghwi; Ryu, Ji Hwa; Kim, Hong Dae [Dept. of Radiology, Haeundae Paik Hospital, Inje University College of Medicine, Busan (Korea, Republic of)

    2014-11-15

    Osmotic demyelination syndrome (ODS), an acquired demyelinating condition of the central pons and/or other regions of the brain, is frequently associated with rapid correction of hyponatremia. There are several reports of ODS in other clinical setting such as malnutrition, alcoholism, transplantation, malignancy, and chronic debilitating illness. However, cases of ODS associated with chemotherapy have not been frequently reported. Here, we describe a case of ODS in a normonatremic patient recently underwent chemotherapy for colon cancer. The diagnosis was confirmed by MRI showing a typical T2 hyperintensity in the central pons. This case suggests that ODS is not always associated with hyponatremia and that ODS can have a favorable clinical and radiologic prognosis.

  3. Anti-Ma2-associated limbic encephalitis with coexisting chronic inflammatory demyelinating polyneuropathy in a patient with non-Hodgkin lymphoma: A case report.

    Science.gov (United States)

    Ju, Weina; Qi, Baochang; Wang, Xu; Yang, Yu

    2017-10-01

    We report the rare case of a 74-year-old man with anti-Ma2-associated paraneoplastic neurologic syndrome (PNS), and review and analyze the clinical manifestations, diagnosis, and treatment of the disease. The patient presented with a 5-month history of muscle weakness, progressive body aches, and weakness and numbness in both lower extremities. Before his hospitalization, he had experienced cognitive function decline; ptosis, inward gaze, and vertical gaze palsy in the right eye; and occasional visual hallucinations. Brain and spinal cord magnetic resonance imaging (MRI) yielded normal results. Anti-Ma2 antibodies were detected in both serum and cerebrospinal fluid. A 4-hour electroencephalogram showed irregular sharp slow waves and δ waves in the temporal region. Electromyography showed peripheral nerve demyelination. Positron-emission tomography/computed tomography (PET-CT) examination revealed hypermetabolism in the lymph nodes of the whole body. Biopsy of the lymph nodes showed non-Hodgkin lymphoma. A clinical diagnosis of lymphoma and PNS was made. The patient was treated with intravenous dexamethasone (15 mg/day) for 3 days. We have presented a rare case of a PNS involving both the central and peripheral nervous systems. The clinical features of this case indicated anti-Ma2-associated encephalitis and chronic inflammatory demyelinating polyneuropathy. PET-CT played a critical role in enabling early diagnosis and prompt treatment in this case.

  4. Anti-Ma2–associated limbic encephalitis with coexisting chronic inflammatory demyelinating polyneuropathy in a patient with non-Hodgkin lymphoma

    Science.gov (United States)

    Ju, Weina; Qi, Baochang; Wang, Xu; Yang, Yu

    2017-01-01

    Abstract Rationale: We report the rare case of a 74-year-old man with anti-Ma2–associated paraneoplastic neurologic syndrome (PNS), and review and analyze the clinical manifestations, diagnosis, and treatment of the disease. Patient concerns: The patient presented with a 5-month history of muscle weakness, progressive body aches, and weakness and numbness in both lower extremities. Before his hospitalization, he had experienced cognitive function decline; ptosis, inward gaze, and vertical gaze palsy in the right eye; and occasional visual hallucinations. Brain and spinal cord magnetic resonance imaging (MRI) yielded normal results. Anti-Ma2 antibodies were detected in both serum and cerebrospinal fluid. A 4-hour electroencephalogram showed irregular sharp slow waves and δ waves in the temporal region. Electromyography showed peripheral nerve demyelination. Positron-emission tomography/computed tomography (PET-CT) examination revealed hypermetabolism in the lymph nodes of the whole body. Biopsy of the lymph nodes showed non-Hodgkin lymphoma. Diagnosis: A clinical diagnosis of lymphoma and PNS was made. Interventions: The patient was treated with intravenous dexamethasone (15 mg/day) for 3 days. Lessons: We have presented a rare case of a PNS involving both the central and peripheral nervous systems. The clinical features of this case indicated anti-Ma2–associated encephalitis and chronic inflammatory demyelinating polyneuropathy. PET-CT played a critical role in enabling early diagnosis and prompt treatment in this case. PMID:28984777

  5. The physical anthropometry, lifestyle habits and blood pressure of people presenting with a first clinical demyelinating event compared to controls: the Ausimmune study.

    Science.gov (United States)

    Ponsonby, Anne-Louise; Lucas, Robyn M; Dear, Keith; van der Mei, Ingrid; Taylor, Bruce; Chapman, Caron; Coulthard, Alan; Dwyer, Terence; Kilpatrick, Trevor J; McMichael, Anthony J; Pender, Michael P; Valery, Patricia C; Williams, David

    2013-11-01

    Lifestyle factors prior to a first clinical demyelinating event (FCD), a disorder often preceding the development of clinically definite multiple sclerosis (MS), have not previously been examined in detail. Past tobacco smoking has been consistently associated with MS. This was a multicentre incident case-control study. Cases (n = 282) were aged 18-59 years with an FCD and resident within one of four Australian centres (from latitudes 27°S to 43°S), from 1 November 2003 to 31 December 2006. Controls (n = 558) were matched to cases on age, sex and study region, without CNS demyelination. Exposures measured included current and past tobacco and marijuana, alcohol and beverage use, physical activity patterns, blood pressure and physical anthropometry. A history of smoking ever was associated with FCD risk (AOR 1.89 (95%CL 1.82, 3.52)). Marijuana use was not associated with FCD risk after adjusting for confounders such as smoking ever but the estimates were imprecise because of a low prevalence of use. Alcohol consumption was common and not associated with FCD risk. No case-control differences in blood pressure or physical anthropometry were observed. Past tobacco smoking was positively associated with a risk of FCD but most other lifestyle factors were not. Prevention efforts against type 2 diabetes and cardiovascular disease by increasing physical activity and reducing obesity are unlikely to alter MS incidence, and more targeted campaigns will be required.

  6. Chronic inflammatory demyelinating polyneuropathy in adults: diagnostic approaches and first line therapy

    Directory of Open Access Journals (Sweden)

    N. А. Suponevа

    2016-01-01

    Full Text Available Chronic inflammatory demyelinating polyneuropathy (CIDP is among the key reasons of chronic polyneuropathies in adults. Diagnostic algorithm of CIDP in adults is presented. Diagnosis of CIDP is based on clinical and electrodiagnostic criteria of European Federation of Neurological Societies/Peripheral Nervous System in 2010. Principles of CIDP treatment are discussed, including modern trends of standard and 10 % IVIG solutions. 

  7. Experimental immunological demyelination enhances regeneration in autograft-repaired long peripheral nerve gaps

    OpenAIRE

    Jun Ge; Shu Zhu; Yafeng Yang; Zhongyang Liu; Xueyu Hu; Liangliang Huang; Xin Quan; Meng Wang; Jinghui Huang; Yunqing Li; Zhuojing Luo

    2016-01-01

    Peripheral nerve long gap defects are a clinical challenge in the regeneration field. Despite the wide variety of surgical techniques and therapies, autografting is the ?gold standard? for peripheral nerve gap reconstruction. The pathological process of Wallerian degeneration from the time of acute injury to efficient regeneration requires several weeks. Regeneration time is critical for nerve reconstruction. Immunological demyelination induced by anti-galactocerebroside antibodies plus guine...

  8. Cisplatin-induced peripheral neuropathy. Frequent off-therapy deterioration, demyelinating syndromes, and muscle cramps.

    Science.gov (United States)

    Siegal, T; Haim, N

    1990-09-15

    Forty-five patients with cisplatin-induced peripheral neuropathy (PN) were evaluated retrospectively after treatment with cumulative doses of cisplatin ranging from 201 to 1952 mg/m2. The patients were followed for up to 23 months (median, 4.5 months), and 32 of them were evaluated more than once. Severity of symptoms was related to higher cumulative doses of cisplatin but with marked individual variability. Off-therapy deterioration of the PN continued in 14 patients (31%) for 2.5 to 5.5 months after withdrawal of cisplatin, and only four patients showed some improvement during the follow-up period. Symptomatic deterioration often was heralded by new onset of muscle cramps (with normal Ca2+/Mg2+ levels) and/or by manifestations of probable spinal dorsal column and/or nerve root demyelinating syndromes presenting as either Lhermitte's sign and/or as an electric-shock sensation along the upper extremities when outstretched in 90 degrees shoulder abduction. Cramps and demyelinating syndromes were each noted in 31% of the patients. Muscle cramps tended to resolve several months after withdrawal of therapy, and demyelinating syndromes were always transient (1.5 to 6.0 months) and did not progress despite ongoing therapy in five patients. Our study indicates that, after withdrawal of therapy, patients with cisplatin-induced PN may continue to deteriorate for several months. Manifestations of muscle cramps and demyelinating syndromes signify a worsening course of the PN but should not automatically indicate interruption of therapy.

  9. Human immunodeficiency virus seroconversion presenting with acute inflammatory demyelinating polyneuropathy: a case report

    Directory of Open Access Journals (Sweden)

    Sloan Derek J

    2008-12-01

    Full Text Available Abstract Introduction Acute Human Immunodeficiency Virus infection is associated with a range of neurological conditions. Guillain-Barré syndrome is a rare presentation; acute inflammatory demyelinating polyneuropathy is the commonest form of Guillain-Barré syndrome. Acute inflammatory demyelinating polyneuropathy has occasionally been reported in acute Immunodeficiency Virus infection but little data exists on frequency, management and outcome. Case presentation We describe an episode of Guillain-Barré syndrome presenting as acute inflammatory demyelinating polyneuropathy in a 30-year-old man testing positive for Immunodeficiency Virus, probably during acute seroconversion. Clinical suspicion was confirmed by cerebrospinal fluid analysis and nerve conduction studies. Rapid clinical deterioration prompted intravenous immunoglobulin therapy and early commencement of highly active anti-retroviral therapy. All symptoms resolved within nine weeks. Conclusion Unusual neurological presentations in previously fit patients are an appropriate indication for Immunodeficiency-Virus testing. Highly active anti-retroviral therapy with adequate penetration of the central nervous system should be considered as an early intervention, alongside conventional therapies such as intravenous immunoglobulin.

  10. Acute Inflammatory Demyelinating Polyneuropathy and a Unilateral Babinski/Plantar Reflex

    Directory of Open Access Journals (Sweden)

    Davide Cattano

    2008-01-01

    Full Text Available Acquired acute demyelinating peripheral polyneuropathy (AADP is a general classification of pathologies that could affect secondary the peripheral nervous system. They are characterized by an autoimmune process directed towards myelin. Clinically they are characterized by progressive weakness and mild sensory changes. Acute inflammatory demyelinating polyneuropathy often is referred to as Guillain-Barré syndrome (GBS. GBS is the major cause of acute nontraumatic paralysis in healthy people and it is caused by autoimmune response to viral agents (influenza, coxsackie, Epstein-Barr virus, or cytomegalovirus or bacterial infective organisms (Campylobacter jejuni, Mycoplasma pneumoniae. A detailed history, with symptoms of progressive usually bilateral weakness, hyporeflexia, with a typical demyelinating EMG pattern supports the diagnosis. Progressive affection of respiratory muscles and autonomic instability coupled with a protracted and unpredictable recovery normally results in the need for ICU management. We present a case report of a patient with a typical GBS presentation but with a unilateral upgoing plantar reflex (Babinski sign. A unifying diagnosis was made and based on a literature search in Pubmed appears to be the first described case of its kind.

  11. Cryptococcal Meningitis Masquerading as Normal Pressure Hydrocephalus in an Immune-competent Adult.

    Science.gov (United States)

    Raheja, Hitesh; Sinha, Ankur; Irukulla, Pavan Kumar; Kupfer, Yizhak

    2017-01-01

    We report a case of acute cryptococcal meningitis (CM) masquerading as normal pressure hydrocephalus (NPH) in an immune-competent female. An 85-year-old human immunodeficiency virus-negative female presented to the emergency room for altered mental status and difficulty walking. She was increasingly lethargic, with urinary incontinence and gait instability. A previous computed tomography was reported to have ventricular dilatation out of proportion to the degree of cortical atrophy. Magnetic resonance scan of the brain revealed ventricular dilatation and subtle debris layering the occipital horns of the lateral ventricles. A working diagnosis of NPH had been made considering the clinical symptoms and imaging. She became febrile to 103°F. Lumbar puncture was then performed which showed increased protein, decreased glucose, and mononuclear pleocytosis. India ink preparation of the cerebrospinal fluid was positive for Cryptococcus along with a positive cryptococcal antigen test. The patient was started on treatment for CM, but the patient continued to deteriorate further and died on the same day. Blood cultures subsequently grew Cryptococcus neoformans as well.

  12. Cryptococcal meningitis masquerading as normal pressure hydrocephalus in an immune-competent adult

    Directory of Open Access Journals (Sweden)

    Hitesh Raheja

    2017-01-01

    Full Text Available We report a case of acute cryptococcal meningitis (CM masquerading as normal pressure hydrocephalus (NPH in an immune-competent female. An 85-year-old human immunodeficiency virus-negative female presented to the emergency room for altered mental status and difficulty walking. She was increasingly lethargic, with urinary incontinence and gait instability. A previous computed tomography was reported to have ventricular dilatation out of proportion to the degree of cortical atrophy. Magnetic resonance scan of the brain revealed ventricular dilatation and subtle debris layering the occipital horns of the lateral ventricles. A working diagnosis of NPH had been made considering the clinical symptoms and imaging. She became febrile to 103°F. Lumbar puncture was then performed which showed increased protein, decreased glucose, and mononuclear pleocytosis. India ink preparation of the cerebrospinal fluid was positive for Cryptococcus along with a positive cryptococcal antigen test. The patient was started on treatment for CM, but the patient continued to deteriorate further and died on the same day. Blood cultures subsequently grew Cryptococcus neoformans as well.

  13. An uncommon variant of double-chambered right ventricle masquerading as double-chambered left ventricle.

    Science.gov (United States)

    Baritakis, Nikolaos; Grapsas, Nikolaos; Kotsalos, Andreas; Davlouros, Periklis

    2018-02-01

    We present a rare case of a double-chambered right ventricle masquerading as a double-chambered left ventricle, which was found incidentally on cardiac imaging in an adult female patient with atypical chest pain. The most common form of double-chambered right ventricle is characterized by compartmentalization of the right ventricle by muscular bands into 2 distinct chambers. The main features of this malformation are a pressure gradient between the 2 compartments, and the frequent (up to 90%) association with a membranous ventricular septal defect. In our case, the muscular band dividing the right ventricle was located in the inferoseptal part of the latter, creating a diminutive cavity that had no communication with the main right ventricle but communicated with the left ventricle creating the false impression of a double-chambered left ventricle. This constitutes a rare variant of double-chambered right ventricle with unknown clinical implications. © The Author 2017. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.

  14. [Masquerade of sebaceous gland carcinoma as a rapidly recurring "chalazion", a case report].

    Science.gov (United States)

    Gerber, D M; Meyer, P; Messerli, J O; Piffaretti, J M; Haefliger, I O

    2001-05-01

    Demonstration of the importance of surgical excision and histological examination in presence of an apparently harmless tumoral alteration of the eyelids. We report the case of a 65 year old patient suffering from systemic lupus erythematosus who noted a tumoral lesion on his left lower eyelid. Suspecting a chalazion, his dermatologist simply performed a cauterization. Six weeks later, a recurrence of the tumor appeared at the same location, and again, cauterization was done. A few weeks later, the patient consulted our clinic with a polycyclic tumor of 5 mm in diameter, involving the lid margin of the temporal part of the lower left eyelid. The patient had only moderate signs of blepharitis. There were no palpable preauricular and cervical lymph nodes. Suspecting a malignant tumor, the entire tumoral lesion was removed surgically. The histopathologic examination showed a highly differentiated sebaceous gland carcinoma, most probably originating from a meibomian gland. The margins of the excision were found to be tumor-free. Sebaceous cell carcinoma is a rare entity. Depending on its histological differentiation it can be highly malignant. Infiltrative, and can metastasize. The mortality may reach 30% if low differentiation is present. As illustrated in the present case, the lesion may masquerade a chalazion. Therefore, in case of atypical lesion of the eyelid region complete surgical removal followed by a histopathological examination should be performed.

  15. Cutaneous squamous cell carcinoma of the eyelid masquerading as a chalazion.

    Science.gov (United States)

    Vrcek, Ivan; Hogan, R N; Mancini, Ronald

    2015-02-01

    Chalazia are among the most common eyelid lesions presenting to eye care providers. Often successfully managed conservatively, some require more invasive intervention such as incision and drainage or steroid injection. Lesions that recur, do not respond to treatment, or are atypical in appearance or natural history should prompt more thorough analysis, often with biopsy and subsequent microscopic analysis. Not uncommonly, such atypical chalazia may be masking a more serious diagnosis. Eyelid cutaneous squamous cell carcinoma masquerading as a chalazion is exceedingly rare. We present a case report of an atypical chalazion that was refractory to incision and drainage as well as intralesional steroid injection. Incisional biopsy revealed the lesion to be a cutaneous squamous cell carcinoma requiring full-thickness excision and subsequent reconstruction. The patient provided written informed consent, and the contents herein are acceptable under the provisions of the institutional review board. Following Mohs excision and oculoplastic reconstruction with a Hughes flap, the patient has had a good outcome and is currently free of recurrence. Recurrent chalazia that are defiant to surgical and medical interventions should prompt biopsy and evaluation by pathology. Cutaneous squamous cell carcinoma should be considered in the differential diagnosis as early intervention can save a patient's eye and, not infrequently, their life.

  16. Giant ovarian cyst masquerading as a massive ascites: a case report.

    Science.gov (United States)

    Yeika, Eugene Vernyuy; Efie, Derrick Tembi; Tolefac, Paul Nkemtendong; Fomengia, Joseph Nkeangu

    2017-12-19

    Giant ovarian cysts are tumours of the ovary presenting with diameters greater than 10 cm. Giant ovarian cysts have become rare in recent days as they are diagnosed and managed early due to the availability of good imaging modalities. The aim of this case report is to show how a huge cystic ovarian mass can mislead the diagnosis of ascites in a postmenopausal woman. Factors associated with late presentation of giant ovarian cysts in sub-Saharan Africa have also been discussed. We present the case of a 65-year-old grand multiparous woman who was referred to our centre with a grossly distended abdomen misdiagnosed as a massive ascites. Abdominopelvic ultrasound scan revealed a right giant multiloculated ovarian cyst. She benefited from a cystectomy with an uneventful postoperative stay. Histopathology revealed mucinous cystadenoma. Large cystic ovarian tumours can present masquerading as massive ascites and misleading diagnosis as in this case report. We report this case to increase the suspicion index of a large ovarian cyst in all women presenting with massive ascites.

  17. High-mass twins & resolution of the reconfinement, masquerade and hyperon puzzles of compact star interiors

    Energy Technology Data Exchange (ETDEWEB)

    Blaschke, David [Bogoliubov Laboratory for Theoretical Physics, JINR Dubna, 141980 Dubna (Russian Federation); Instytut Fizyki Teoretycznej, Uniwersytet Wroclawski, 50-204 Wroclaw (Poland); Alvarez-Castillo, David E. [Bogoliubov Laboratory for Theoretical Physics, JINR Dubna, 141980 Dubna (Russian Federation); Instituto de Física, Universidad Autónoma de San Luis Potosí, San Luis Potosí, México (Mexico)

    2016-01-22

    We aim at contributing to the resolution of three of the fundamental puzzles related to the still unsolved problem of the structure of the dense core of compact stars (CS): (i) the hyperon puzzle: how to reconcile pulsar masses of 2 M{sub ⊙} with the hyperon softening of the equation of state (EoS); (ii) the masquerade problem: modern EoS for cold, high density hadronic and quark matter are almost identical; and (iii) the reconfinement puzzle: what to do when after a deconfinement transition the hadronic EoS becomes favorable again? We show that taking into account the compositeness of baryons (by excluded volume and/or quark Pauli blocking) on the hadronic side and confining and stiffening effects on the quark matter side results in an early phase transition to quark matter with sufficient stiffening at high densities which removes all three present-day puzzles of CS interiors. Moreover, in this new class of EoS for hybrid CS falls the interesting case of a strong first order phase transition which results in the observable high mass twin star phenomenon, an astrophysical observation of a critical endpoint in the QCD phase diagram.

  18. Lateral rectus metastasis from an occult systemic malignancy masquerading as abducens palsy: a case report

    Directory of Open Access Journals (Sweden)

    Masoud Mohammad T

    2008-06-01

    Full Text Available Abstract Introduction Abduction deficit in the elderly is commonly caused by sixth cranial nerve palsy due to microvasculopathy. However, not all such cases are of neurogenic origin, as our case report shows. Case presentation We present the case of a 75-year-old woman who was generally unwell, developed acute diplopia and was found to have a right abduction deficit in a quiet eye with no gross orbital signs and symptoms. A computed tomography scan of the head and orbits revealed a metastatic mass in the right lateral rectus muscle. Systemic evaluation confirmed widespread thoracic and abdominal metastases from an occult systemic malignancy. Lateral rectus metastasis from an occult systemic malignancy was masquerading as abducens palsy. Conclusion Orbital metastasis involving extraocular muscles can present as isolated diplopia with minimal local signs and the absence of a history of systemic malignancy. A detailed history and systemic examination can identify suspicious cases, which should be investigated further. The clinician should avoid presuming that such an abduction deficit in the elderly is a benign neurogenic palsy.

  19. Targeting demyelination and virtual hypoxia with high-dose biotin as a treatment for progressive multiple sclerosis.

    Science.gov (United States)

    Sedel, Frédéric; Bernard, Delphine; Mock, Donald M; Tourbah, Ayman

    2016-11-01

    Progressive multiple sclerosis (MS) is a severely disabling neurological condition, and an effective treatment is urgently needed. Recently, high-dose biotin has emerged as a promising therapy for affected individuals. Initial clinical data have shown that daily doses of biotin of up to 300 mg can improve objective measures of MS-related disability. In this article, we review the biology of biotin and explore the properties of this ubiquitous coenzyme that may explain the encouraging responses seen in patients with progressive MS. The gradual worsening of neurological disability in patients with progressive MS is caused by progressive axonal loss or damage. The triggers for axonal loss in MS likely include both inflammatory demyelination of the myelin sheath and primary neurodegeneration caused by a state of virtual hypoxia within the neuron. Accordingly, targeting both these pathological processes could be effective in the treatment of progressive MS. Biotin is an essential co-factor for five carboxylases involved in fatty acid synthesis and energy production. We hypothesize that high-dose biotin is exerting a therapeutic effect in patients with progressive MS through two different and complementary mechanisms: by promoting axonal remyelination by enhancing myelin production and by reducing axonal hypoxia through enhanced energy production. This article is part of the Special Issue entitled 'Oligodendrocytes in Health and Disease'. Copyright © 2015 The Authors. Published by Elsevier Ltd.. All rights reserved.

  20. Quetiapine, an atypical antipsychotic, is protective against autoimmune-mediated demyelination by inhibiting effector T cell proliferation.

    Directory of Open Access Journals (Sweden)

    Feng Mei

    Full Text Available Quetiapine (Que, a commonly used atypical antipsychotic drug (APD, can prevent myelin from breakdown without immune attack. Multiple sclerosis (MS, an autoimmune reactive inflammation demyelinating disease, is triggered by activated myelin-specific T lymphocytes (T cells. In this study, we investigated the potential efficacy of Que as an immune-modulating therapeutic agent for experimental autoimmune encephalomyelitis (EAE, a mouse model for MS. Que treatment was initiated on the onset of MOG(35-55 peptide induced EAE mice and the efficacy of Que on modulating the immune response was determined by Flow Cytometry through analyzing CD4(+/CD8(+ populations and the proliferation of effector T cells (CD4(+CD25(- in peripheral immune organs. Our results show that Que dramatically attenuates the severity of EAE symptoms. Que treatment decreases the extent of CD4(+/CD8(+ T cell infiltration into the spinal cord and suppresses local glial activation, thereby diminishing the loss of mature oligodendrocytes and myelin breakdown in the spinal cord of EAE mice. Our results further demonstrate that Que treatment decreases the CD4(+/CD8(+ T cell populations in lymph nodes and spleens of EAE mice and inhibits either MOG(35-55 or anti-CD3 induced proliferation as well as IL-2 production of effector T cells (CD4(+CD25(- isolated from EAE mice spleen. Together, these findings suggest that Que displays an immune-modulating role during the course of EAE, and thus may be a promising candidate for treatment of MS.

  1. Western Theatre and the Production Processes of Ọkumkpọ Masquerade Performance of Akpọha-Afikpo: A Study in Comparison

    Directory of Open Access Journals (Sweden)

    Bernard Eze Orji

    2017-09-01

    Full Text Available Masquerade performances transcend mere mask adornment. It involves preparations that take varying lengths of time and painstaking efforts. During the elaborate behind-the-scene pre-performance processes of mask and costume construction, musical and choreography practices, composition of new songs and tunes, making of props and floats; all participants showcase deep gestalt spirit and collaboration. Such energies are also found within the process of production in contemporary western theatre. Way beyond arguing whether masquerade performances qualify as theatre or not, this paper concerns itself with x-raying the procedures of staging Ọkumkpọ Masquerade of Akpọha, and determining how each of its segments (script consultation, audition, rehearsals, building and or hiring costumes, publicity, presentation exhibit theatreness. The preferred tool for this enquiry is performance analysis because of its suitability for interrogating each individual aspect of a staged/live event. Interviews will also be employed as an auxiliary method for eliciting data from participants. This paper concludes that every aspect of the modern theatre directing reflects in Ọkumkpọ Masquerade theatre production processes.

  2. A missed diagnosis or a masquerading disease: back to the basics ...

    African Journals Online (AJOL)

    Due to her labile blood pressures despite intravenous hydralazine and metoprolol, the pregnancy was terminated. However, she continued to have labile blood pressures. Better control of blood pressure was achieved on oral prazocin and nifedipine. The patient was then transferred to floor and discharged home a few days ...

  3. Visceral larval migrans masquerading as metastatic disease in a toddler with Wilms tumor

    International Nuclear Information System (INIS)

    Anderson, Andrew; Fordham, Lynn Ansley; Bula, Melania L.; Blatt, Julie

    2006-01-01

    A 22-month-old girl with a renal mass had multiple small pulmonary nodules on CT at her initial presentation. After biopsy and neoadjuvant chemotherapy, a Wilms tumor was resected and the pulmonary nodules were shown to have regressed on CT. Follow-up imaging 4 months after initial diagnosis demonstrated multiple new liver lesions and new pulmonary nodules with peripheral eosinophilia. Lung biopsy revealed granuloma formation with prominent eosinophils. The serum antibody titers for Toxocara canis were elevated. This case illustrates that toxocariasis should be considered as a rare differential diagnostic possibility for multiple liver lesions and multifocal peripheral pulmonary opacities in young children with Wilms tumor. (orig.)

  4. Visceral larval migrans masquerading as metastatic disease in a toddler with Wilms tumor

    Energy Technology Data Exchange (ETDEWEB)

    Anderson, Andrew; Fordham, Lynn Ansley; Bula, Melania L. [University of North Carolina School of Medicine, Department of Radiology, Chapel Hill, NC (United States); Blatt, Julie [University of North Carolina School of Medicine, Department of Pediatrics, Chapel Hill, NC (United States)

    2006-03-15

    A 22-month-old girl with a renal mass had multiple small pulmonary nodules on CT at her initial presentation. After biopsy and neoadjuvant chemotherapy, a Wilms tumor was resected and the pulmonary nodules were shown to have regressed on CT. Follow-up imaging 4 months after initial diagnosis demonstrated multiple new liver lesions and new pulmonary nodules with peripheral eosinophilia. Lung biopsy revealed granuloma formation with prominent eosinophils. The serum antibody titers for Toxocara canis were elevated. This case illustrates that toxocariasis should be considered as a rare differential diagnostic possibility for multiple liver lesions and multifocal peripheral pulmonary opacities in young children with Wilms tumor. (orig.)

  5. Progressive Multiple Sclerosis patients show substantial lesion activity that correlates with clinical disease severity and sex: a retrospective autopsy cohort analysis.

    NARCIS (Netherlands)

    Luchetti, S.; Fransen, N.L.; van Eden, C.G.; Ramaglia, V.; Mason, M.; Huitinga, I.

    2018-01-01

    Multiple sclerosis (MS) is a highly heterogeneous disease with large inter-individual differences in disease course. MS lesion pathology shows considerable heterogeneity in localization, cellular content and degree of demyelination between patients. In this study, we investigated pathological

  6. The endocannabinoid system and its therapeutic exploitation in multiple sclerosis : Clues for other neuroinflammatory diseases.

    NARCIS (Netherlands)

    Chiurchiù, V.; Stelt, van der M.; Centonze, D.; Maccarrone, M.

    2017-01-01

    Multiple sclerosis is the most common inflammatory demyelinating disease of the central nervous system, caused by an autoimmune response against myelin that eventually leads to progressive neurodegeneration and disability. Although the knowledge on its underlying neurobiological mechanisms has

  7. PET Imaging of Disease Progression and Treatment Effects in the Experimental Autoimmune Encephalomyelitis Rat Model

    NARCIS (Netherlands)

    Faria, Daniele de Paula; Vlaming, Maria L. H.; Copray, Sjef C. V. M.; Tielen, Frans; Anthonijsz, Herma J. A.; Sijbesma, Jurgen W. A.; Buchpiguel, Carlos A.; Dierckx, Rudi A. J. O.; van der Hoorn, Jose W. A.; de Vries, Erik F. J.

    The experimental autoimmune encephalomyelitis model is a model of multiple sclerosis that closely mimics the disease characteristics in humans. The main hallmarks of multiple sclerosis are neuroinflammation (microglia activation, monocyte invasion, and T-cell infiltration) and demyelination. PET

  8. Retinal nerve fibre layer thinning is associated with worse visual outcome after optic neuritis in children with a relapsing demyelinating syndrome.

    Science.gov (United States)

    Eyre, Michael; Hameed, Aasim; Wright, Sukhvir; Brownlee, Wallace; Ciccarelli, Olga; Bowman, Richard; Lim, Ming; Wassmer, Evangeline; Thompson, Dorothy; Hemingway, Cheryl; Hacohen, Yael

    2018-04-11

    Optic neuritis may be monophasic or occur as part of a relapsing demyelinating syndrome (RDS), such as multiple sclerosis, aquaporin-4 antibody (AQP4-Ab) neuromyelitis optical spectrum disorder (NMOSD), or myelin oligodendrocyte glycoprotein antibody (MOG-Ab)-associated disease. The aims of this study were to test whether clinical, electrophysiological, and microstructural parameters differ in multiple-sclerosis-associated optic neuritis (MS-ON) and antibody-associated optic neuritis (Ab-ON); to identify the clinical and paraclinical characteristics of children suffering worse long-term visual outcome of RDS-optic neuritis; and to explore the relationship between RNFL thickness and clinical parameters in RDS-optic neuritis. Forty-two children with optic neuritis were retrospectively studied: 22 with multiple sclerosis (MS-ON) and 20 with antibody-associated demyelination (Ab-ON: MOG-Ab=16 and AQP4-Ab=4). Clinical and paraclinical features were analysed. Complete recovery of visual acuity was reported in 25 out of 42 children; eight out of 38 (21%) suffered moderate or severe visual impairment (logarithm of the minimum angle of resolution [logMAR]>0.5) in their worse eye, including four out of 38 who were blind (logMAR>1.3) in their worse eye (two with multiple sclerosis, two with AQP4-Ab NMOSD). None of the children with MOG-Ab were blind. Recurrence of optic neuritis was more common in the Ab-ON group than the MS-ON group (15 out of 20 vs seven out of 22, p=0.007). Retinal nerve fibre layer (RNFL) thickness at baseline inversely correlated with visual acuity at final follow-up (r=-0.41, p=0.008). There was no significant relationship between the number of episodes of optic neuritis and mean RNFL (r=-0.08, p=0.628), nor any significant relationship between the number of episodes of optic neuritis and visual impairment (r=0.03, p=0.794). In children with RDS, long-term visual impairment inversely correlated with RNFL thickness, but not with the number of relapses of

  9. A new pattern of spinal-cord demyelination in guinea pigs with acute experimental allergic encephalomyelitis mimicking multiple sclerosis.

    Science.gov (United States)

    Colover, J.

    1980-01-01

    A technique is described for producing large demyelinating lesions of the spinal cord in the guinea pig. Guinea pigs were pretreated by immunization with ovalbumin and water-soluble adjuvant (N-acetyl-muramyl L-alanyl D-isoglutamine, MDP) in water-in-oil emulsion (Freund's incomplete adjuvant). They were given a large dose (10 mg) of ovalbumin i.p. one month later. After a few weeks the animals were sensitized with guinea-pig basic protein in Freund's complete adjuvant. Five out of 11 animals developed large, distinctive, sharply demarcated, symmetrical demyelinating lesions within 30 days. These lesions occurred in the dorsal and anterior columns, root entry zones and subpial region of the spinal cord. Histology showed a considerable amount of free lipids. There were also infiltrative lesions of classical experimental allergic encephalomyelitis (EAE) of normal severity in the same animals. The demyelinating lesions resembled those seen in multiple sclerosis in their location and extent in the spinal cord and in the presence of free lipids. Control experiments indicated that pretreatment with ovalbumin/MDP and the second injection of ovalbumin was necessary for all the demyelination; moreover guinea pigs immunized with basic protein in Freund's complete adjuvant or Freund's incomplete adjuvant plus MDP without pretreatment only developed classical EAE with minimal or no demyelination. Images Fig. 1 Fig. 2 Fig. 3 Fig. 4 Fig. 5 Fig. 6 Fig. 7 PMID:7426390

  10. Guillain-Barre syndrome masquerading as acute respiratory failure in an infant.

    Science.gov (United States)

    Kishore, Praveen; Sharma, Pradeep Kumar; Saikia, Bhaskar; Khilnani, Praveen

    2015-01-01

    Guillain-Barré syndrome (GBS) is a rare entity in infants. We report a case of GBS in a 5-month-old girl. The child presented with cough, loose stools, breathing difficulty, and listlessness. The child was treated as pneumonia with respiratory failure. Due to difficulty in weaning from ventilation with areflexia, marked hypotonia, and reduced power in all four limbs; possibilities of spinal muscular atrophy, poliomyelitis, and myopathies were kept. Nerve conduction velocity study was suggestive of mixed sensory-motor, severe axonal, and demyelinating polyradiculoneuropathy. Cerebrospinal fluid study revealed albuminocytological dissociation. Child was diagnosed as GBS and treated with intravenous immunoglobulin. Child recovered completely on follow-up. GBS should be considered as a differential diagnosis in acute onset respiratory failure with neuromuscular weakness in infants.

  11. Ghost maculopathy: an artifact on near-infrared reflectance and multicolor imaging masquerading as chorioretinal pathology.

    Science.gov (United States)

    Pang, Claudine E; Freund, K Bailey

    2014-07-01

    To describe the features of an artifact on near-infrared reflectance and MultiColor imaging, termed "ghost maculopathy," and to illustrate how it may masquerade as true chorioretinal pathology. This was a retrospective, observational case series. The authors studied 144 eyes of 72 consecutive patients in a vitreoretinal clinical practice, reviewing multimodal imaging including color and red-free fundus photography, fundus autofluorescence (FAF), near-infrared reflectance, MultiColor imaging, and spectral-domain optical coherence tomography (SD OCT). In 36 of 144 eyes (25%), there was an appearance of a hyper-reflective spot on near-infrared reflectance and MultiColor imaging, located at the macula, nasal or superonasal to the fovea, which did not correspond to any apparent lesion on color and red-free fundus photography, FAF, or SD OCT. This spot was termed the "ghost image" in this phenomenon of "ghost maculopathy." The ghost image was present consistently on near-infrared reflectance and MultiColor imaging in all 36 eyes at every imaging encounter, showing minimal and subtle variability in its shape and location within each eye; however, it showed large interindividual variability in size, shape, location, and reflectivity between different eyes. Nine eyes were found to have a similar hyper-reflective spot resembling that in ghost maculopathy, but corresponding SD OCT images were consistent with diagnoses of choroidal nevus, age-related macular degeneration, and multifocal choroiditis. All eyes with ghost maculopathy were found to be pseudophakic with a posterior chamber intraocular lens. Ghost maculopathy is the phenomenon of an imaging artifact appearing at the macula on near-infrared reflectance and MultiColor imaging that occurs predominantly in pseudophakic patients and may be mistaken for true chorioretinal pathology. Awareness of this artifact is prudent to avoid misinterpretation of clinical findings and possible unnecessary over-investigation. Copyright

  12. Neuromyelitis optica-IgG testing in an Indian cohort with neuromyelitis optica and related demyelinating disorders: Our experience

    Directory of Open Access Journals (Sweden)

    Narayanan Unni

    2013-01-01

    Full Text Available Background: Neuromyelitis optica (NMO is an immune-mediated inflammatory demyelinating disorder of the central nervous system with a predilection for the optic nerves and the spinal cord. Immunopathological evidence suggests that the target antigen of the disease is aquaporin-4. An IgG antibody against this protein has been explored as a molecular marker for the disease and as a diagnostic tool due to its high sensitivity and specificity in various populations. Objective: To assess the value of NMO-IgG testing in Indian patients with clinical and magnetic resonance imaging features consistent with NMO and longitudinally extensive transverse myelitis (LETM. Materials and Methods: Forty-five patients with clinical and magnetic resonance imaging features consistent with NMO, LETM, and MS were tested for serum NMO-IgG. Of these patients, 22 patients satisfied revised (2006 Wingerchuk criteria for NMO (excluding NMO-IgG status and 11 patients had LETM. Twelve patients satisfied the revised (2010 McDonald criteria for multiple sclerosis (MS. Results: Of the 21 patients, satisfying the criteria for NMO and for whom the test results were available, 17 were positive for NMO-IgG (80.9%, and of the 11 patients having LETM, 6 (54.5% were positive for NMO-IgG. In one patient with NMO, the test result was not available. None of the 12 patients satisfying McDonald criteria for MS showed NMO-IgG seropositivity. Conclusion: Our study suggests that it is worthwhile to pursue NMO-IgG testing as a diagnostic tool for patients with clinical and Magnetic Resonance Imaging (MRI features consistent with NMO and LETM in the Indian population.

  13. Abnormal scintigrams in demyclinating diseases of the brain

    International Nuclear Information System (INIS)

    Podreka, I.; Heiss, W.D.; Jellinger, K.; Vienna Univ.

    1977-01-01

    In 6 patients with acute or exacerbating demyelinating disease and in 2 cases suffering from adrenoleucodystrophy (Schilder's disease) brain scintigraphy revealed areas with increased isotope uptake. The pathological foci were verified by autopsy in 4 cases and by inspection in 1. These foci are difficult to distinguish from other localized brain diseases causing increased isotope uptake; in addition to the clinical course and to spinal fluid abnormalities reversibility of the scintigraphic lesions indicate demyelinating diseases. The typical pattern of symmetrical lesions within the white matter and the progression of abnormalities from the occipital to the frontal lobe speak in favor of Schilder's disease. (orig.) [de

  14. Diffusion Magnetic Resonance Imaging May Provide Prognostic Information in Osmotic Demyelination Syndrome: Report of a Case

    Energy Technology Data Exchange (ETDEWEB)

    Dervisoglu, E.; Yegenaga, I.; Anik, Y.; Sengul, E.; Turgut, T. [Kocaeli Univ. (Turkey). Internal Medicine

    2006-03-15

    Hyponatremia and its rapid correction may cause osmotic demyelination syndrome (ODS) with damage to the pontine and extrapontine areas of the brain. The damage may become persistent or may regress and disappear during follow-up. We describe the case of a 35-year-old woman with chronic renal failure who was admitted to the emergency department with profound hyponatremia which was corrected rapidly after hemodialysis treatment. During follow-up, she developed quadriparesis and dysartria. Magnetic resonance imaging (MRI) demonstrated abnormalities characteristic of ODS in the pons as well as the basal ganglia with increased signal intensity on T2 and diffusion-weighted (DW) MRI and low apparent diffusion coefficient (ADC) values. After the sixth day, her clinical status improved progressively. Control MRI revealed rapid normalization of the ADC values during the first week and month parallel to the clinical improvement. However, the hyperintensities on T2-weighted images persisted. Four months later the MRI findings were completely normal. The close relationship between the ADC abnormality and the clinical status suggests that DW-MRI may be useful in predicting the prognosis of ODS. Keywords: Apparent diffusion coefficient; correction of hyponatremia; magnetic resonance imaging; osmotic demyelination.

  15. Experimental demyelination and axonal loss are reduced in MicroRNA-146a deficient mice

    DEFF Research Database (Denmark)

    Martin, Nellie A.; Molnar, Viktor; Szilagyi, Gabor T.

    2018-01-01

    Background: The cuprizone (CPZ) model of multiple sclerosis (MS) was used to identify microRNAs (miRNAs) related to in vivo de- and remyelination. We further investigated the role of miR-146a in miR-146a-deficient (KO) mice: this miRNA is differentially expressed in MS lesions and promotes...... chromatography tandem mass spectrometry) of CPZ-induced de- and remyelination in WT mice. Levels of proinflammatory molecules in the corpus callosum were compared in WT versus KO mice by Meso Scale Discovery multiplex protein analysis. Results: miR-146a was increasingly upregulated during CPZ-induced de...... mice. miR-146a target genes, SNAP25 and SMAD4, were downregulated in the proteome of demyelinating corpus callosum in WT mice. Higher levels of SNAP25 were measured by ELISA in the corpus callosum of miR-146a KO mice, but there was no difference between KO and WT mice during demyelination. Multiplex...

  16. Comparison of electrodiagnostic abnormalities and criteria in a cohort of patients with chronic inflammatory demyelinating polyneuropathy.

    Science.gov (United States)

    Magda, Paul; Latov, Norman; Brannagan, Thomas H; Weimer, Louis H; Chin, Russell L; Sander, Howard W

    2003-12-01

    Current electrodiagnostic criteria for chronic inflammatory demyelinating polyneuropathy (CIDP) are research oriented favoring specificity over sensitivity. Application of such criteria in clinical practice may miss the diagnosis in potentially treatable patients. To analyze the electrophysiologic abnormalities in a cohort of patients with clinically defined CIDP, to compare these data with published electrodiagnostic criteria, and to identify a set of abnormalities that is shared by all patients with CIDP. Retrospective medical record review. Academically based neuromuscular clinic. Patients Fifteen patients with clinically diagnosed relapsing sensorimotor CIDP. Administration of intravenous immunoglobulin or prednisone. Electrodiagnostic studies. All patients had electrodiagnostic abnormalities in at least 3 nerves with possible partial conduction block or demyelinating range abnormalities in at least 1 nerve. The diagnostic sensitivities of 5 published CIDP criteria were as follows: the Ad Hoc Subcommittee of the American Academy of Neurology AIDS Task Force (40%), Saperstein et al (47%), Nicolas et al (53%), Hughes et al for the Inflammatory Neuropathy Cause and Treatment Group (60%), and Thaisetthawatkul et al (70%). Current electrodiagnostic criteria for CIDP are insensitive and may fail to diagnose the condition in a substantial number of patients. More inclusive criteria that allow identification of patients in routine clinical practice are needed.

  17. Role of Demyelination Efficiency within Acellular Nerve Scaffolds during Nerve Regeneration across Peripheral Defects

    Directory of Open Access Journals (Sweden)

    Meiqin Cai

    2017-01-01

    Full Text Available Hudson’s optimized chemical processing method is the most commonly used chemical method to prepare acellular nerve scaffolds for the reconstruction of large peripheral nerve defects. However, residual myelin attached to the basal laminar tube has been observed in acellular nerve scaffolds prepared using Hudson’s method. Here, we describe a novel method of producing acellular nerve scaffolds that eliminates residual myelin more effectively than Hudson’s method through the use of various detergent combinations of sulfobetaine-10, sulfobetaine-16, Triton X-200, sodium deoxycholate, and peracetic acid. In addition, the efficacy of this new scaffold in repairing a 1.5 cm defect in the sciatic nerve of rats was examined. The modified method produced a higher degree of demyelination than Hudson’s method, resulting in a minor host immune response in vivo and providing an improved environment for nerve regeneration and, consequently, better functional recovery. A morphological study showed that the number of regenerated axons in the modified group and Hudson group did not differ. However, the autograft and modified groups were more similar in myelin sheath regeneration than the autograft and Hudson groups. These results suggest that the modified method for producing a demyelinated acellular scaffold may aid functional recovery in general after nerve defects.

  18. Further insight on A-wave in acute and chronic demyelinating neuropathies.

    Science.gov (United States)

    Sartucci, Ferdinando; Bocci, Tommaso; Borghetti, Davide; Orlandi, Giovanni; Manfredonia, Francesco; Murri, Luigi; Giannini, Fabio; Rossi, Alessandro

    2010-10-01

    A-wave is a late motor response that maintains the same characteristics of latency, amplitude and shape with every electrical stimuli at a proper given intensity. The presence of A-waves was reported both in chronic (CIDP) and acute (AIDP) forms of inflammatory demyelinating polyradiculoneuropathy. It is attributed to the effect of either sprouting phenomena or ephaptic/ectopic discharge. In the first condition it could be a sign of functional recovery, while in the second it could represent an early indicator of demyelination. Aims of our research were to investigate retrospectively the presence of the A-waves, establishing whether its frequency is more common in CIDP or AIDP. Data from 77 patients, 57 male and 20 female, mean age 60.7 years (SD 15.4), were recovered from clinical records and their neurophysiological tests retrieved for reanalysis. Our results seem to indicate that A-waves can represent an early sign of acute pathology of peripheral nervous system.

  19. Antibody response against gastrointestinal antigens in demyelinating diseases of the central nervous system

    DEFF Research Database (Denmark)

    Banati, M; Csecsei, P; Koszegi, E

    2013-01-01

    BACKGROUND: Antibodies against gastrointestinal antigens may indicate altered microbiota and immune responses in the gut. Recent experimental data suggest a connection between gastrointestinal immune responses and CNS autoimmunity. METHODS: Antibodies against gliadin, tissue transglutaminase (tTG...

  20. Using atypical symptoms and red flags to identify non-demyelinating disease.

    LENUS (Irish Health Repository)

    Kelly, Siobhan B

    2012-01-01

    Red flags and atypical symptoms have been described as being useful in suggesting alternative diagnoses to multiple sclerosis (MS) and clinically isolated syndrome (CIS); however, their diagnostic utility has not been assessed. The aim of this study was to establish the predictive value of red flags and the typicality\\/atypicality of symptoms at presentation in relation to the final diagnosis of patients referred with suspected MS.

  1. Marchiafava - bignami disease: a case report.

    Science.gov (United States)

    Kumar K, Sunil; Challam, Rimiki; J, Naveen; Singh, W Jatishwor

    2014-08-01

    Marchiafava-Bignami disease (MBD) is a rare neurological disorder of chronic alcoholism characterized by demyelination and necrosis of corpus callosum. In this case report we present the MR imaging findings of MBD and review of the other imaging features of the disease.

  2. Neuroantigen-specific autoregulatory CD8+ T cells inhibit autoimmune demyelination through modulation of dendritic cell function.

    Directory of Open Access Journals (Sweden)

    Venkatesh P Kashi

    Full Text Available Experimental autoimmune encephalomyelitis (EAE is a well-established murine model of multiple sclerosis, an immune-mediated demyelinating disorder of the central nervous system (CNS. We have previously shown that CNS-specific CD8+ T cells (CNS-CD8+ ameliorate EAE, at least in part through modulation of CNS-specific CD4+ T cell responses. In this study, we show that CNS-CD8+ also modulate the function of CD11c+ dendritic cells (DC, but not other APCs such as CD11b+ monocytes or B220+ B cells. DC from mice receiving either myelin oligodendrocyte glycoprotein-specific CD8+ (MOG-CD8+ or proteolipid protein-specific CD8+ (PLP-CD8+ T cells were rendered inefficient in priming T cell responses from naïve CD4+ T cells (OT-II or supporting recall responses from CNS-specific CD4+ T cells. CNS-CD8+ did not alter DC subset distribution or MHC class II and CD86 expression, suggesting that DC maturation was not affected. However, the cytokine profile of DC from CNS-CD8+ recipients showed lower IL-12 and higher IL-10 production. These functions were not modulated in the absence of immunization with CD8-cognate antigen, suggesting an antigen-specific mechanism likely requiring CNS-CD8-DC interaction. Interestingly, blockade of IL-10 in vitro rescued CD4+ proliferation and in vivo expression of IL-10 was necessary for the suppression of EAE by MOG-CD8+. These studies demonstrate a complex interplay between CNS-specific CD8+ T cells, DC and pathogenic CD4+ T cells, with important implications for therapeutic interventions in this disease.

  3. Anti-S-nitrosocysteine antibodies are a predictive marker for demyelination in experimental autoimmune encephalomyelitis: implications for multiple sclerosis.

    Science.gov (United States)

    Boullerne, Anne I; Rodriguez, Jose J; Touil, Tarik; Brochet, Bruno; Schmidt, Stephan; Abrous, Nora D; Le Moal, Michel; Pua, Jeffrey R; Jensen, Mark A; Mayo, Willy; Arnason, Barry G W; Petry, Klaus G

    2002-01-01

    Multiple sclerosis (MS) is characterized by inflammation within the CNS. This inflammatory response is associated with production of nitric oxide (NO) and NO-related species that nitrosylate thiols. We postulated that MS patients would exhibit an antibody (Ab) response directed against proteins containing S-nitrosocysteine (SNO-cysteine) and showed that anti-NO-cysteine Abs of the IgM isotype are in fact present in the sera of some MS patients (Boullerne et al., 1995). We report here the presence of a seemingly identical Ab response directed against SNO-cysteine in an acute model of MS, experimental autoimmune encephalomyelitis (EAE) induced in Lewis rats with the 68-84 peptide of guinea pig myelin basic protein (MBP(68-84)). Serum levels of anti-SNO-cysteine Abs peaked 1 week before the onset of clinical signs and well before the appearance of anti-MBP(68-84) Abs. The anti-SNO-cysteine Ab peak titer correlated with the extent of subsequent CNS demyelination, suggesting a link between Ab level and CNS lesion formation. In relapsing-remitting MS patients, we found elevated anti-SNO-cysteine Ab at times of relapse and normal values in most patients judged to be in remission. Two-thirds of patients with secondary progressive MS had elevated anti-SNO-cysteine Ab levels, including those receiving interferon beta-1b. The data show that a rise in circulating anti-SNO-cysteine Ab levels precedes onset of EAE. Anti-SNO-cysteine Abs are also elevated at times of MS attacks and in progressive disease, suggesting a possible role for these Abs, measurable in blood, as a biological marker for clinical activity.

  4. Intravenous immunoglobulin treatment in chronic inflammatory demyelinating polyradiculoneuropathy, a time to start and a time to stop

    NARCIS (Netherlands)

    Adrichem, Max E.; Eftimov, Filip; van Schaik, Ivo N.

    2016-01-01

    Intravenous immunoglobulin (IVIg) is often used as preferred treatment in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Several studies highlighted the short-term efficacy of IVIg for CIDP yet many patients need maintenance therapy. Notwithstanding the fact IVIg has been used for

  5. p75(NTR) independent oligodendrocyte death in cuprizone-induced demyelination in C57BL/6 mice

    NARCIS (Netherlands)

    Copray, JCVM; Kust, BM; Mantingh-Otter, [No Value; Boddeke, HWGM

    2005-01-01

    Feeding C57Bl/6 J mice the copper chelator cuprizone leads to selective apoptosis of mature oligodendrocytes and concomitant demyelination predominantly in the corpus callosum. The process of oligodendrocyte apoptosis in this animal model for multiple sclerosis (MS) involves early microglial

  6. A comparative study of brachial plexus sonography and magnetic resonance imaging in chronic inflammatory demyelinating neuropathy and multifocal motor neuropathy

    NARCIS (Netherlands)

    Goedee, H S; Jongbloed, B A; van Asseldonk, J.T.H.; Hendrikse, J; Vrancken, A F J E; Franssen, H; Nikolakopoulos, S; Visser, L H; van der Pol, W L; van den Berg, L H

    2017-01-01

    BACKGROUND AND PURPOSE: To compare the performance of neuroimaging techniques, i.e. high-resolution ultrasound (HRUS) and magnetic resonance imaging (MRI), when applied to the brachial plexus, as part of the diagnostic work-up of chronic inflammatory demyelinating neuropathy (CIDP) and multifocal

  7. The Protective Effects of Areca catechu Extract on Cognition and Social Interaction Deficits in a Cuprizone-Induced Demyelination Model

    Directory of Open Access Journals (Sweden)

    Abulimiti Adilijiang

    2015-01-01

    Full Text Available Schizophrenia is a serious psychiatric illness with an unclear cause. One theory is that demyelination of white matter is one of the main pathological factors involved in the development of schizophrenia. The current study evaluated the protective effects of Areca catechu nut extract (ANE on a cuprizone-induced demyelination mouse model. Two doses of ANE (1% and 2% were administered orally in the diet for 8 weeks. Animals subjected to demyelination showed impaired spatial memory and less social activity. In addition, mice subjected to demyelination displayed significant myelin damage in cortex and demonstrated a higher expression of NG2 and PDGFRα and AMPK activation. ANE treatment not only significantly enhanced cognitive ability and social activity, but also protected myelin against cuprizone toxicity by promoting oligodendrocyte precursor cell (OPC differentiation. In addition, ANE treatment demonstrated significant dephosphorylation of AMPKα, indicating a regulatory role for ANE in schizophrenia. This study showed that ANE treatment may enhance cognitive ability and social activity by facilitating OPC differentiation and protecting against myelin damage in cortex. Results also suggest the AMPK signaling pathway may be involved in this process.

  8. The masquerade game: marine mimicry adaptation between egg-cowries and octocorals

    Directory of Open Access Journals (Sweden)

    Juan A. Sánchez

    2016-08-01

    -fertilize. Discussion. Our study system comprised background-matching mimicry, of the masquerade type, between egg-cowries (Simnia/Simnialena and octocorals (Pacifigorgia/Eugorgia/Leptogorgia. We observed mimicry mismatches related to fitness trade-offs, such as reproductive aggregations vs. vulnerability against predators. Despite the general assumption that coevolution of mimicry involves speciation, egg-cowries with different hosts and colorations comprise the same lineages. Consequently, we infer that there would be significant tradeoffs between mimicry and the pursuit of reproductive aggregations in egg-cowries. The findings of this study not only contribute to the understanding of the evolution of mimicry in egg-cowries, a poorly studied group of marine gastropods, but also to the development of a new biologically meaningful board game that could be implemented as a learning tool.

  9. Spatial and temporal profiles of growth factor expression during CNS demyelination reveal the dynamics of repair priming.

    Directory of Open Access Journals (Sweden)

    Viktoria Gudi

    Full Text Available Demyelination is the cause of disability in various neurological disorders. It is therefore crucial to understand the molecular regulation of oligodendrocytes, the myelin forming cells in the CNS. Growth factors are known to be essential for the development and maintenance of oligodendrocytes and are involved in the regulation of glial responses in various pathological conditions. We employed the well established murine cuprizone model of toxic demyelination to analyze the expression of 13 growth factors in the CNS during de- and remyelination. The temporal mRNA expression profile during demyelination and the subsequent remyelination were analyzed separately in the corpus callosum and cerebral cortex using laser microdissection and real-time PCR techniques. During demyelination a similar pattern of growth factor mRNA expression was observed in both areas with a strong up-regulation of NRG1 and GDNF and a slight increase of CNTF in the first week of cuprizone treatment. HGF, FGF-2, LIF, IGF-I, and TGF-ß1 were up-regulated mainly during peak demyelination. In contrast, during remyelination there were regional differences in growth factor mRNA expression levels. GDNF, CNTF, HGF, FGF-2, and BDNF were elevated in the corpus callosum but not in the cortex, suggesting tissue differences in the molecular regulation of remyelination in the white and grey matter. To clarify the cellular source we isolated microglia from the cuprizone lesions. GDNF, IGF-1, and FGF mRNA were detected in the microglial fraction with a temporal pattern corresponding to that from whole tissue PCR. In addition, immunohistochemical analysis revealed IGF-1 protein expression also in the reactive astrocytes. CNTF was located in astrocytes. This study identified seven different temporal expression patterns for growth factors in white and grey matter and demonstrated the importance of early tissue priming and exact orchestration of different steps during callosal and cortical de

  10. The Prevalence and Severity of Autonomic Dysfunction in Chronic Inflammatory Demyelinating Polyneuropathy.

    Science.gov (United States)

    Pasangulapati, Suresh Babu; Murthy, T V; Sivadasan, Ajith; Gideon, L Rynjah; Prabhakar, A T; Sanjith, Aaron; Mathew, Vivek; Alexander, Mathew

    2017-01-01

    In chronic inflammatory demyelinating polyneuropathy (CIDP), emphasis has been on motor disabilities, and autonomic dysfunction in these patients has not been addressed systematically. Autonomic function was prospectively analyzed in 38 patients with CIDP. Quantitative autonomic function testing was done using Finometer ® PRO and severity of adrenergic and cardiovagal dysfunction graded according to composite autonomic severity score and sudomotor dysfunction assessed using sympathetic skin response. Thirty-four (89%) patients had features of autonomic dysfunction. Thirty-three (86%) patients had cardiovagal dysfunction, 21 (55%) had adrenergic dysfunction, and 24 (63%) had sudomotor dysfunction. Autonomic dysfunction was mild to moderate in the majority (86%). Autonomic dysfunction in CIDP is underreported and potentially amenable to therapy. Our cohort had a high proportion of adrenergic dysfunction compared to previous studies.

  11. Changes in spatiotemporal gait parameters following intravenous immunoglobulin treatment for chronic inflammatory demyelinating polyneuropathy.

    Science.gov (United States)

    Vo, Mary L; Chin, Russell L; Miranda, Caroline; Latov, Norman

    2017-10-01

    Gait impairment is a common presenting symptom in patients with chronic inflammatory demyelinating polyneuropathy (CIDP). However, gait parameters have not previously been evaluated in detail as potential independent outcome measures. We prospectively measured changes in spatiotemporal gait parameters of 20 patients with CIDP at baseline and following treatment with intravenous immunoglobulin (IVIG), using GAITRite® a computerized walkway system with embedded sensors. Overall, study patients showed significant improvements in gait velocity, cadence, stride length, double support time, stance phase, and swing phase following IVIG treatment. Mean changes in velocity, stance phase, and swing phase, exhibited the greatest statistical significance among the subgroup that exhibited clinically meaningful improvement in Inflammatory Neuropathy Cause and Treatment disability score, Medical Research Council sum score, and grip strength. Assessment of gait parameters, in particular velocity, step phase and swing phase, is a potentially sensitive outcome measure for evaluating treatment response in CIDP. Muscle Nerve 56: 732-736, 2017. © 2017 Wiley Periodicals, Inc.

  12. Noninvasive Detection and Differentiation of Axonal Injury/Loss, Demyelination, and Inflammation

    Science.gov (United States)

    2015-10-01

    spectrum imaging, diffusion tensor imaging, EAE, inflammation, axonal injury, curizone, demyelination, optic neuritis, axonal loss 16. SECURITY...Std. Z39.18 34 - 3 - Table of Contents Page 1. Introduction …………………………………………………………. 4 2. Keywords……………………………………………………………. 4 3. Overall Project...6 10. References………………………………………………………….. 6 – 7 11. Appendices…………………………………………………………. 8 - 4 - Introduction Acute neurological

  13. Resistance training and aerobic training improve muscle strength and aerobic capacity in chronic inflammatory demyelinating polyneuropathy

    DEFF Research Database (Denmark)

    Markvardsen, Lars H; Overgaard, Kristian; Heje, Karen

    2018-01-01

    INTRODUCTION: We investigated the effects of aerobic and resistance exercise in patients with chronic inflammatory demyelinating polyneuropathy (CIDP). METHODS: Eighteen CIDP patients treated with subcutaneous immunoglobulin performed 12 weeks of aerobic exercise and 12 weeks of resistance exercise...... after a run-in period of 12 weeks without exercise. Three times weekly the participants performed aerobic exercise on an ergometer bike or resistance exercise with unilateral training of knee and elbow flexion/extension. Primary outcomes were maximal oxygen consumption velocity (VO2 -max) and maximal...... resulted in an increase of 13.8% ± 16.0% (P = 0.0004) in cIKS. DISCUSSION: Aerobic exercise training and resistance exercise training improve fitness and strength in CIDP patients. Muscle Nerve, 2017....

  14. Trigeminal Nerve Root Demyelination Not Seen in Six Horses Diagnosed with Trigeminal-Mediated Headshaking

    Directory of Open Access Journals (Sweden)

    Veronica L. Roberts

    2017-05-01

    Full Text Available Trigeminal-mediated headshaking is an idiopathic neuropathic facial pain syndrome in horses. There are clinical similarities to trigeminal neuralgia, a neuropathic facial pain syndrome in man, which is usually caused by demyelination of trigeminal sensory fibers within either the nerve root or, less commonly, the brainstem. Our hypothesis was that the neuropathological substrate of headshaking in horses is similar to that of trigeminal neuralgia in man. Trigeminal nerves, nerve roots, ganglia, infraorbital, and caudal nasal nerves from horse abattoir specimens and from horses euthanized due to trigeminal-mediated headshaking were removed, fixed, and processed for histological assessment by a veterinary pathologist and a neuropathologist with particular experience of trigeminal neuralgia histology. No histological differences were detected between samples from horses with headshaking and those from normal horses. These results suggest that trigeminal-mediated headshaking may have a different pathological substrate from trigeminal neuralgia in man.

  15. An analysis of autopsy cases of non-Hodgkin lymphoma-with special reference to those masquerading as acute febrile illness

    Directory of Open Access Journals (Sweden)

    Sonali Rajesh Saraf

    2016-01-01

    Conclusion: As NHL present with nonspecific symptoms, these tumours may not be detected in early stages and hence may not be treated appropriately. These patients have weakened immunity and hence are prone to infection and sepsis which can be a major cause of mortality. This autopsy study experience has shown that NHL can masquerade as acute febrile illness which if not detected early and treated adequately can turn fatal.

  16. Comparison of electrophysiological findings in axonal and demyelinating Guillain-Barre syndrome

    Science.gov (United States)

    Yadegari, Samira; Nafissi, Shahriar; Kazemi, Neda

    2014-01-01

    Background: Incidence and predominant subtype of Guillain-Barre syndrome (GBS) differs geographically. Electrophysiology has an important role in early diagnosis and prediction of prognosis. This study is conducted to determine the frequent subtype of GBS in a large group of patients in Iran and compare nerve conduction studies in axonal and demyelinating forms of GBS. Methods: We retrospectively evaluated the medical records and electrodiagnostic study (EDS) of 121 GBS patients who were managed in our hospital during 11 years. After regarding the exclusion criteria, patients classified as three groups: acute inflammatory demyelinating polyneuropathy (AIDP), acute motor axonal neuropathy (AMAN), and acute motor sensory axonal neuropathy (AMSAN). The most frequent subtype and then electrophysiological characteristic based on the time of EDS and their cerebrospinal fluid (CSF) profile were assessed. Results: Among 70 patients finally included in the study, 67% were men. About 63%, 23%, and 14% had AIDP, AMAN, and AMSAN, respectively. AIDP patients represented a wider range of ages compared with other groups. Higher levels of CSF protein, abnormal late responses and sural sparing were more frequent in AIDP subtype. Five AMSAN patients also revealed sural sparing. Conduction block (CB) was observed in one AMAN patient. Prolonged F-wave latency was observed only in AIDP cases. CB and inexcitable sensory nerves were more frequent after 2 weeks, but reduced F-wave persistency was more prominent in the early phase. Conclusion: AIDP was the most frequent subtype. Although the electrophysiology and CSF are important diagnostic tools, classification should not be made based on a distinct finding. PMID:25422732

  17. Lipoprotein Lipase Is a Feature of Alternatively-Activated Microglia and May Facilitate Lipid Uptake in the CNS During Demyelination.

    Science.gov (United States)

    Bruce, Kimberley D; Gorkhali, Sachi; Given, Katherine; Coates, Alison M; Boyle, Kristen E; Macklin, Wendy B; Eckel, Robert H

    2018-01-01

    Severe demyelinating disorders of the central nervous system (CNS) such as multiple sclerosis (MS), can be devastating for many young lives. To date, the factors resulting in poor remyelination and repair are not well understood, and reparative therapies that benefit MS patients have yet to be developed. We have previously shown that the activity and abundance of Lipoprotein Lipase (LPL)-the rate-limiting enzyme in the hydrolysis of triglyceride-rich lipoproteins-is increased in Schwann cells and macrophages following nerve crush injury in the peripheral nervous system (PNS), suggesting that LPL may help scavenge myelin-derived lipids. We hypothesized that LPL may play a similar role in the CNS. To test this, mice were immunized with MOG 35-55 peptide to induce experimental allergic encephalomyelitis (EAE). LPL activity was increased ( p < 0.05) in the brain at 30 days post-injection, coinciding with partial remission of clinical symptoms. Furthermore, LPL abundance and activity was up-regulated ( p < 0.05) at the transition between de- and re-myelination in lysolecithin-treated ex vivo cerebellar slices. Since microglia are the key immune effector cells of the CNS we determined the role of LPL in microglia. Lipid uptake was decreased ( p < 0.001) in LPL-deficient BV-2 microglial cells compared to WT. In addition, LPL-deficient cells showed dramatically reduced expression of anti-inflammatory markers, YM1 (-22 fold, p < 0.001), and arginase 1 (Arg1; -265 fold, p < 0.001) and increased expression of pro-inflammatory markers, such as iNOS compared to WT cells (+53 fold, p < 0.001). This suggests that LPL is a feature of reparative microglia, further supported by the metabolic and inflammatory profile of LPL-deficient microglia. Taken together, our data strongly suggest that LPL expression is a novel feature of a microglial phenotype that supports remyelination and repair through the clearance of lipid debris. This mechanism may be exploited to develop future

  18. Biology of the repair of central nervous system demyelinated lesions: an appraisal Biologia da reparação de lesões desmielinizantes do sistema nervoso central: uma avaliação

    Directory of Open Access Journals (Sweden)

    L. A. V Peireira

    1996-06-01

    Full Text Available The integrity of myelin sheaths is maintained by oligodendrocytes and Schwann cells respectively in the central nervous system (CNS and in the peripheral nervous system. The process of demyelination consisting of the withdrawal of myelin sheaths from their axons is a characteristic feature of multiple sclerosis, the most common human demyelinating disease. Many experimental models have been designed to study the biology of demyelination and remyelination (repair of the lost myelin in the CNS, due to the difficulties in studying human material. In the ethidium bromide (an intercalating gliotoxic drug model of demyelination, CNS remyelination may be carried out by surviving oligodendrocytes and/or by cells differentiated from the primitive cell lines or either by Schwann cells that invade the CNS. However, some factors such as the age of the experimental animals, intensity and time of exposure to the intercalating chemical and the topography of the lesions have marked influence on the repair of the tissue.A integridade da bainha de mielina é fornecida pelos oligodendrócitos e pelas células de Schwann, no sistema nervoso central (SNC e no sistema nervoso periférico, respectivamente. O fenômeno de desmielinização refere-se à remoção das bainhas de mielina de axônios e este fato é característico na esclerose múltipla, a doença desmielinizante do SNC mais comum no homem. Muitos modelos experimentais têm sido utilizados para o estudo da biologia da desmielinização e remielinização no SNC, face à dificuldade de estudo de material humano. No modelo experimental da droga intercalate, gliotóxica, brometo de etídio, a remielinização do SNC pode ser efetuada por oligodendrócitos sobreviventes à lesão e/ou oriundos de diferenciação de linhagens celulares mais primitivas e por células de Schwann que invadem o SNC. No entanto, fatores como a idade dos animais, a intensidade, e o tempo de exposição ao agente intercalante e a

  19. A recurrence of Guillain-Barr and eacute; syndrome or a case of acute-onset chronic inflammatory demyelinating polyneuropathy in the course of chronic hepatitis B?

    Directory of Open Access Journals (Sweden)

    Guner Celik Koyuncu

    2016-12-01

    Full Text Available Chronic inflammatory demyelinating polyneuropathy is a demyelinating polyneuropathy characterized by distal/proximal weakness, which shows gradual progression over a period of 8 weeks or longer. Guillan-Barre Syndrome is a condition characterized by acute monophasic paralysis typically following an infectious assault, and it usually peaks in severity over 3-4 weeks at most. Although rare, there are acute-onset chronic inflammatory demyelinating polyneuropathy cases that show progression over a period shorter than 4 weeks, as is the case in Guillan-Barre Syndrome .This report discusses a case of chronic inflammatory demyelinating polyneuropathy in a HBsAg-positive patient, which started as Guillan-Barre Syndrome but showed 3 recurrences within 6 months, each with rapidly progressing quadriplegia, respiratory arrest, and elevated liver enzymes and HBV DNA. [Cukurova Med J 2016; 41(4.000: 782-786

  20. A randomised, double-blinded, placebo-controlled trial of the effect of subcuta-neous immunoglobulin on muscular performance in chronic inflammatory de-myelinating polyneuropathy

    DEFF Research Database (Denmark)

    Harbo, Thomas; Markvardsen, Lars Høj; Sindrup, Søren Hein

    Objectives: Subcutaneous treatment with large amounts of immunoglobulins is feasible and effective in multifocal motor neuropathy and has been reported in a few cases in chronic inflammatory demyelinating polyneuropathy (CIDP). We hypothesized that the effect of subcutaneous treatment with immuno...

  1. Central Nervous System Idiopathic Inflammatory Demyelinating Disorders in South Americans: A Descriptive, Multicenter, Cross-Sectional Study.

    Science.gov (United States)

    Papais-Alvarenga, Regina Maria; Vasconcelos, Claudia Cristina Ferreira; Carra, Adriana; de Castillo, Ibis Soto; Florentin, Sara; Diaz de Bedoya, Fernando Hamuy; Mandler, Raul; de Siervi, Luiza Campanella; Pimentel, Maria Lúcia Vellutini; Alvarenga, Marina Papais; Alvarenga, Marcos Papais; Grzesiuk, Anderson Kuntz; Gama Pereira, Ana Beatriz Calmon; Gomes Neto, Antonio Pereira; Velasquez, Carolina; Soublette, Carlos; Fleitas, Cynthia Veronica; Diniz, Denise Sisteroli; Armas, Elizabeth; Batista, Elizabeth; Hernandez, Freda; Pereira, Fernanda Ferreira Chaves da Costa; Siqueira, Heloise Helena; Cabeça, Hideraldo; Sanchez, Jose; Brooks, Joseph Bruno Bidin; Gonçalves, Marcus Vinicius; Barroso, Maria Cristina Del Negro; Ravelo, Maria Elena; Castillo, Maria Carlota; Ferreira, Maria Lúcia Brito; Rocha, Maria Sheila Guimarães; Parolin, Monica Koncke Fiuza; Molina, Omaira; Marinho, Patricia Beatriz Christino; Christo, Paulo Pereira; Brant de Souza, Renata; Pessanha Neto, Silvio; Camargo, Solange Maria das Graças; Machado, Suzana Costa; Neri, Vanderson Carvalho; Fragoso, Yara Dadalti; Alvarenga, Helcio; Thuler, Luiz Claudio Santos

    2015-01-01

    The idiopathic inflammatory demyelinating disease (IIDD) spectrum has been investigated among different populations, and the results have indicated a low relative frequency of neuromyelitis optica (NMO) among multiple sclerosis (MS) cases in whites (1.2%-1.5%), increasing in Mestizos (8%) and Africans (15.4%-27.5%) living in areas of low MS prevalence. South America (SA) was colonized by Europeans from the Iberian Peninsula, and their miscegenation with natives and Africans slaves resulted in significant racial mixing. The current study analyzed the IIDD spectrum in SA after accounting for the ethnic heterogeneity of its population. A cross-sectional multicenter study was performed. Only individuals followed in 2011 with a confirmed diagnosis of IIDD using new diagnostic criteria were considered eligible. Patients' demographic, clinical and laboratory data were collected. In all, 1,917 individuals from 22 MS centers were included (73.7% female, 63.0% white, 28.0% African, 7.0% Mestizo, and 0.2% Asian). The main disease categories and their associated frequencies were MS (76.9%), NMO (11.8%), other NMO syndromes (6.5%), CIS (3.5%), ADEM (1.0%), and acute encephalopathy (0.4%). Females predominated in all main categories. The white ethnicity also predominated, except in NMO. Except in ADEM, the disease onset occurred between 20 and 39 years old, early onset in 8.2% of all cases, and late onset occurred in 8.9%. The long-term morbidity after a mean disease time of 9.28±7.7 years was characterized by mild disability in all categories except in NMO, which was scored as moderate. Disease time among those with MS was positively correlated with the expanded disability status scale (EDSS) score (r=0.374; p=<0.001). This correlation was not observed in people with NMO or those with other NMO spectrum disorders (NMOSDs). Among patients with NMO, 83.2% showed a relapsing-remitting course, and 16.8% showed a monophasic course. The NMO-IgG antibody tested using indirect

  2. Central Nervous System Idiopathic Inflammatory Demyelinating Disorders in South Americans: A Descriptive, Multicenter, Cross-Sectional Study.

    Directory of Open Access Journals (Sweden)

    Regina Maria Papais-Alvarenga

    Full Text Available The idiopathic inflammatory demyelinating disease (IIDD spectrum has been investigated among different populations, and the results have indicated a low relative frequency of neuromyelitis optica (NMO among multiple sclerosis (MS cases in whites (1.2%-1.5%, increasing in Mestizos (8% and Africans (15.4%-27.5% living in areas of low MS prevalence. South America (SA was colonized by Europeans from the Iberian Peninsula, and their miscegenation with natives and Africans slaves resulted in significant racial mixing. The current study analyzed the IIDD spectrum in SA after accounting for the ethnic heterogeneity of its population. A cross-sectional multicenter study was performed. Only individuals followed in 2011 with a confirmed diagnosis of IIDD using new diagnostic criteria were considered eligible. Patients' demographic, clinical and laboratory data were collected. In all, 1,917 individuals from 22 MS centers were included (73.7% female, 63.0% white, 28.0% African, 7.0% Mestizo, and 0.2% Asian. The main disease categories and their associated frequencies were MS (76.9%, NMO (11.8%, other NMO syndromes (6.5%, CIS (3.5%, ADEM (1.0%, and acute encephalopathy (0.4%. Females predominated in all main categories. The white ethnicity also predominated, except in NMO. Except in ADEM, the disease onset occurred between 20 and 39 years old, early onset in 8.2% of all cases, and late onset occurred in 8.9%. The long-term morbidity after a mean disease time of 9.28±7.7 years was characterized by mild disability in all categories except in NMO, which was scored as moderate. Disease time among those with MS was positively correlated with the expanded disability status scale (EDSS score (r=0.374; p=<0.001. This correlation was not observed in people with NMO or those with other NMO spectrum disorders (NMOSDs. Among patients with NMO, 83.2% showed a relapsing-remitting course, and 16.8% showed a monophasic course. The NMO-IgG antibody tested using indirect

  3. Nogo-A is a reliable oligodendroglial marker in adult human and mouse CNS and in demyelinated lesions

    DEFF Research Database (Denmark)

    Kuhlmann, Tanja; Remington, Leah; Maruschak, Brigitte

    2007-01-01

    to be strongly expressed in mature oligodendrocytes in vivo. In the present investigation we analyzed the expression patterns of Nogo-A in adult mouse and human CNS as well as in demyelinating animal models and multiple sclerosis lesions. Nogo-A expression was compared with that of other frequently used...... oligodendroglial markers such as CC1, CNP, and in situ hybridization for proteolipid protein mRNA. Nogo-A strongly and reliably labeled oligodendrocytes in the adult CNS as well as in demyelinating lesions and thus represents a valuable tool for the identification of oligodendrocytes in human and mouse CNS tissue......The unambiguous identification of oligodendrocytes in tissue sections, especially in myelinated tracts, is often difficult. Most of the antibodies used to identify oligodendrocytes label the myelin sheath as well. Originally described as an inhibitor of axonal outgrowth, Nogo-A is known...

  4. Methylcobalamin promotes the differentiation of Schwann cells and remyelination in lysophosphatidylcholine-induced demyelination of the rat sciatic nerve

    Directory of Open Access Journals (Sweden)

    Shunsuke eNishimoto

    2015-08-01

    Full Text Available Schwann cells (SCs are constituents of the peripheral nervous system. The differentiation of SCs in injured peripheral nerves is critical for regeneration after injury. Methylcobalamin (MeCbl is a vitamin B12 analog that is necessary for the maintenance of the peripheral nervous system. In this study, we estimated the effect of MeCbl on SCs. We showed that MeCbl downregulated the activity of Erk1/2 and promoted the expression of the myelin basic protein in SCs. In a dorsal root ganglion neuron–SC coculture system, myelination was promoted by MeCbl. In a focal demyelination rat model, MeCbl promoted remyelination and motor and sensory functional regeneration. MeCbl promoted the in vitro differentiation of SCs and in vivo myelination in a rat demyelination model and may be a novel therapy for several types of nervous disorders.

  5. Relapsing and Progressive Tumefactive Demyelinating Form of Central Nervous System Involvement in a Patient with Progressive Systemic Sclerosis

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Ho Kyun [Dept. of Radiology, Catholic University of Daegu School of Medicine, Daegu (Korea, Republic of); Lee, Hui Joong [Dept. of Kyungpook National University Hospital, Daegu (Korea, Republic of)

    2013-03-15

    White matter hyper intensities (WMHI) on MRI are not rare in patients with progressive systemic sclerosis (PSS). In this presentation, WMHI were developed in both middle cerebellar peduncles and temporal white matter in a patient with PSS, and regressed after medication of high dose steroid. However, new lesions were developed in the subcortices of both precentral gyri, and progressed rapidly to tumefactive hyperintensity on MRI. We report an unusual relapsing and progressive tumefactive demyelinating form of central nervous system involvement in PSS.

  6. Relapsing and Progressive Tumefactive Demyelinating Form of Central Nervous System Involvement in a Patient with Progressive Systemic Sclerosis

    International Nuclear Information System (INIS)

    Kim, Ho Kyun; Lee, Hui Joong

    2013-01-01

    White matter hyper intensities (WMHI) on MRI are not rare in patients with progressive systemic sclerosis (PSS). In this presentation, WMHI were developed in both middle cerebellar peduncles and temporal white matter in a patient with PSS, and regressed after medication of high dose steroid. However, new lesions were developed in the subcortices of both precentral gyri, and progressed rapidly to tumefactive hyperintensity on MRI. We report an unusual relapsing and progressive tumefactive demyelinating form of central nervous system involvement in PSS.

  7. Interactions Between the Canonical WNT/Beta-Catenin Pathway and PPAR Gamma on Neuroinflammation, Demyelination, and Remyelination in Multiple Sclerosis.

    Science.gov (United States)

    Vallée, Alexandre; Vallée, Jean-Noël; Guillevin, Rémy; Lecarpentier, Yves

    2018-05-01

    Multiple sclerosis (MS) is marked by neuroinflammation and demyelination with loss of oligodendrocytes in the central nervous system. The immune response is regulated by WNT/beta-catenin pathway in MS. Activated NF-kappaB, a major effector of neuroinflammation, and upregulated canonical WNT/beta-catenin pathway positively regulate each other. Demyelinating events present an upregulation of WNT/beta-catenin pathway, whereas proper myelinating phases show a downregulation of WNT/beta-catenin pathway essential for the promotion of oligodendrocytes precursors cells proliferation and differentiation. The activation of WNT/beta-catenin pathway results in differentiation failure and impairment in remyelination. However, PI3K/Akt pathway and TCF7L2, two downstream targets of WNT/beta-catenin pathway, are upregulated and promote proper remyelination. The interactions of these signaling pathways remain unclear. PPAR gamma activation can inhibit NF-kappaB, and can also downregulate the WNT/beta-catenin pathway. PPAR gamma and canonical WNT/beta-catenin pathway act in an opposite manner. PPAR gamma agonists appear as a promising treatment for the inhibition of demyelination and the promotion of proper remyelination through the control of both NF-kappaB activity and canonical WNT/beta-catenin pathway.

  8. Treatments for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP): an overview of systematic reviews.

    Science.gov (United States)

    Oaklander, Anne Louise; Lunn, Michael Pt; Hughes, Richard Ac; van Schaik, Ivo N; Frost, Chris; Chalk, Colin H

    2017-01-13

    Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a chronic progressive or relapsing and remitting disease that usually causes weakness and sensory loss. The symptoms are due to autoimmune inflammation of peripheral nerves. CIPD affects about 2 to 3 per 100,000 of the population. More than half of affected people cannot walk unaided when symptoms are at their worst. CIDP usually responds to treatments that reduce inflammation, but there is disagreement about which treatment is most effective. To summarise the evidence from Cochrane systematic reviews (CSRs) and non-Cochrane systematic reviews of any treatment for CIDP and to compare the effects of treatments. We considered all systematic reviews of randomised controlled trials (RCTs) of any treatment for any form of CIDP. We reported their primary outcomes, giving priority to change in disability after 12 months.Two overview authors independently identified published systematic reviews for inclusion and collected data. We reported the quality of evidence using GRADE criteria. Two other review authors independently checked review selection, data extraction and quality assessments.On 31 October 2016, we searched the Cochrane Database of Systematic Reviews, the Database of Abstracts of Reviews of Effects (in theCochrane Library), MEDLINE, Embase, and CINAHL Plus for systematic reviews of CIDP. We supplemented the RCTs in the existing CSRs by searching on the same date for RCTs of any treatment of CIDP (including treatment of fatigue or pain in CIDP), in the Cochrane Neuromuscular Specialised Register, CENTRAL, MEDLINE, Embase, and CINAHL Plus. Five CSRs met our inclusion criteria. We identified 23 randomised trials, of which 15 had been included in these CSRs. We were unable to compare treatments as originally planned, because outcomes and outcome intervals differed. CorticosteroidsIt is uncertain whether daily oral prednisone improved impairment compared to no treatment because the quality of the

  9. Acute-onset chronic inflammatory demyelinating polyneuropathy with cranial nerve involvement, dysautonomia, respiratory failure, and autoantibodies.

    Science.gov (United States)

    Hantson, Philippe; Kevers, Luc; Fabien, Nicole; Van Den Bergh, Peter

    2010-03-01

    We examined a 27-year-old woman who developed rapidly progressive quadriplegia and acute respiratory failure that required mechanical ventilation in the intensive care unit. It was unclear whether this was a presentation of Guillain-Barré syndrome (GBS) or acute-onset chronic inflammatory demyelinating polyradiculoneuropathy (A-CIDP). Remarkable features included multiple cranial nerve involvement, respiratory failure, dysautonomia, and skin manifestations. Several autoantibodies were elevated, including antinuclear (ANA), anticardiolipin (aCL), thyroid, and calcium-sensing receptor (CaSR) autoantibodies. The patient was initially diagnosed with GBS and treated with intravenous immunoglobulin (IVIg). After almost complete recovery, relapse with quadriplegia and respiratory failure was observed 12 weeks after motor symptom onset. She then received IVIg and steroid pulse therapy followed by maintenance oral methylprednisolone and plasma exchange. She recovered completely 4 months after the relapse. The further clinical and serological course was consistent with systemic lupus erythematosus (SLE)-associated CIDP. Herein we evaluate the association between A-CIDP and some biological markers of autoimmunity.

  10. Standing postural reaction to visual and proprioceptive stimulation in chronic acquired demyelinating polyneuropathy

    Directory of Open Access Journals (Sweden)

    Clement P. Provost

    2018-01-01

    Full Text Available Objective: To investigate the weight of visual and proprioceptive inputs, measured indirectly in standing position control, in patients with chronic acquired demyelinating polyneuropathy (CADP. Design: Prospective case study. Subjects: Twenty-five patients with CADP and 25 healthy controls. Methods: Posture was recorded on a double force platform. Stimulations were optokinetic (60°/s for visual input and vibration (50 Hz for proprioceptive input. Visual stimulation involved 4 tests (upward, downward, rightward and leftward and proprioceptive stimulation 2 tests (triceps surae and tibialis anterior. A composite score, previously published and slightly modified, was used for the recorded postural signals from the different stimulations. Results: Despite their sensitivity deficits, patients with CADP were more sensitive to proprioceptive stimuli than were healthy controls (mean composite score 13.9 ((standard deviation; SD 4.8 vs 18.4 (SD 4.8, p = 0.002. As expected, they were also more sensitive to visual stimuli (mean composite score 10.5 (SD 8.7 vs 22.9 (SD 7.5, p< 0.0001. Conclusion: These results encourage balance rehabilitation of patients with CADP, aimed at promoting the use of proprioceptive information, thereby reducing too-early development of visual compensation while proprioception is still available.

  11. Intrahepatic Cholangiocarcinoma Masquerading as Acute Fatty Liver of Pregnancy: A Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Ayman Qasrawi

    2018-01-01

    Full Text Available Cholangiocarcinoma (CCA is an uncommon cancer and accounts only for 3% of all gastrointestinal malignancies. In this report, we present a case of an intrahepatic cholangiocarcinoma masquerading as acute fatty liver of pregnancy (AFLP. A 38-year-old female who is 36-week pregnant presented with a 1-week history of headache, nausea, vomiting, and right upper abdominal pain, along with hepatomegaly. Laboratory investigations were remarkable for mild leukocytosis, hyperbilirubinemia, proteinuria, and elevated transaminases and prothrombin time. Ultrasound of the liver revealed hepatomegaly, fatty infiltration, and a right hepatic lobe mass. Based on the overall picture, AFLP was suspected, and the patient underwent delivery by Cesarean section. However, bilirubin and liver enzyme levels gradually increased after delivery. MRI revealed a large dominant hepatic mass along with multiple satellite lesions in both lobes. Biopsy revealed the presence of intrahepatic CCA. CCA presenting during pregnancy is extremely rare with only 9 other cases reported in the literature. Therefore, the signs and symptoms can be easily confused with other more common disorders that occur during pregnancy.

  12. Combining Diffusion Tensor Metrics and DSC Perfusion Imaging: Can It Improve the Diagnostic Accuracy in Differentiating Tumefactive Demyelination from High-Grade Glioma?

    Science.gov (United States)

    Hiremath, S B; Muraleedharan, A; Kumar, S; Nagesh, C; Kesavadas, C; Abraham, M; Kapilamoorthy, T R; Thomas, B

    2017-04-01

    Tumefactive demyelinating lesions with atypical features can mimic high-grade gliomas on conventional imaging sequences. The aim of this study was to assess the role of conventional imaging, DTI metrics ( p:q tensor decomposition), and DSC perfusion in differentiating tumefactive demyelinating lesions and high-grade gliomas. Fourteen patients with tumefactive demyelinating lesions and 21 patients with high-grade gliomas underwent brain MR imaging with conventional, DTI, and DSC perfusion imaging. Imaging sequences were assessed for differentiation of the lesions. DTI metrics in the enhancing areas and perilesional hyperintensity were obtained by ROI analysis, and the relative CBV values in enhancing areas were calculated on DSC perfusion imaging. Conventional imaging sequences had a sensitivity of 80.9% and specificity of 57.1% in differentiating high-grade gliomas ( P = .049) from tumefactive demyelinating lesions. DTI metrics ( p : q tensor decomposition) and DSC perfusion demonstrated a statistically significant difference in the mean values of ADC, the isotropic component of the diffusion tensor, the anisotropic component of the diffusion tensor, the total magnitude of the diffusion tensor, and rCBV among enhancing portions in tumefactive demyelinating lesions and high-grade gliomas ( P ≤ .02), with the highest specificity for ADC, the anisotropic component of the diffusion tensor, and relative CBV (92.9%). Mean fractional anisotropy values showed no significant statistical difference between tumefactive demyelinating lesions and high-grade gliomas. The combination of DTI and DSC parameters improved the diagnostic accuracy (area under the curve = 0.901). Addition of a heterogeneous enhancement pattern to DTI and DSC parameters improved it further (area under the curve = 0.966). The sensitivity increased from 71.4% to 85.7% after the addition of the enhancement pattern. DTI and DSC perfusion add profoundly to conventional imaging in differentiating tumefactive

  13. Subcutaneous immunoglobulin in responders to intravenous therapy with chronic inflammatory demyelinating polyradiculoneuropathy.

    Science.gov (United States)

    Markvardsen, L H; Debost, J-C; Harbo, T; Sindrup, S H; Andersen, H; Christiansen, I; Otto, M; Olsen, N K; Lassen, L L; Jakobsen, J

    2013-05-01

    We hypothesized that subcutaneous administration of immunoglobulins (SCIG) in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is feasible, safe and superior to treatment with saline for the performance of muscle strength. Thirty patients with motor involvement in maintenance therapy with intravenous immunoglobulin (IVIG) fulfilling the EFNS/PNS criteria for CIDP, aged 18-80 years, were randomized either to SCIG at a dose corresponding to their pre-study IVIG dose or to subcutaneous saline given twice or thrice weekly for 12 weeks at home. At the start and end of the trial as well as 2 weeks before (-2, 0, 10, 12 weeks), isokinetic strength performance of four predetermined and weakened muscle groups was measured. Also, an Overall Disability Sum Score (ODSS), 40-m-walking test (40-MWT), nine-hole-peg test, Neurological Impairment Score (NIS), Medical Research Council (MRC) score, grip strength, standardized electrophysiological recordings from three nerves, and plasma IgG levels were evaluated. SCIG treatment was well tolerated in all 14 patients. Six patients complained of mild side-effects at the injection site. In the SCIG group there was an increase of isokinetic muscle strength of 5.5 ± 9.5% (P < 0.05) as compared with a decline of 14.4 ± 20.3% (P < 0.05) in the placebo group; the difference between the two groups being significant (P < 0.01). ODSS, NIS, MRC, grip strength and 40-MWT improved following SCIG versus saline. SCIG treatment in CIDP is feasible, safe and effective, and seems an attractive alternative to IVIG. © 2013 The Author(s) European Journal of Neurology © 2013 EFNS.

  14. Diffusion Tensor Imaging as a Biomarker to Differentiate Acute Disseminated Encephalomyelitis From Multiple Sclerosis at First Demyelination.

    Science.gov (United States)

    Aung, Wint Yan; Massoumzadeh, Parinaz; Najmi, Safa; Salter, Amber; Heaps, Jodi; Benzinger, Tammie L S; Mar, Soe

    2018-01-01

    There are no clinical features or biomarkers that can reliably differentiate acute disseminated encephalomyelitis from multiple sclerosis at the first demyelination attack. Consequently, a final diagnosis is sometimes delayed by months and years of follow-up. Early treatment for multiple sclerosis is recommended to reduce long-term disability. Therefore, we intend to explore neuroimaging biomarkers that can reliably distinguish between the two diagnoses. We reviewed prospectively collected clinical, standard MRI and diffusion tensor imaging data from 12 pediatric patients who presented with acute demyelination with and without encephalopathy. Patients were followed for an average of 6.5 years to determine the accuracy of final diagnosis. Final diagnosis was determined using 2013 International Pediatric MS Study Group criteria. Control subjects consisted of four age-matched healthy individuals for each patient. The study population consisted of six patients with central nervous system demyelination with encephalopathy with a presumed diagnosis of acute disseminated encephalomyelitis and six without encephalopathy with a presumed diagnosis of multiple sclerosis or clinically isolated syndrome at high risk for multiple sclerosis. During follow-up, two patients with initial diagnosis of acute disseminated encephalomyelitis were later diagnosed with multiple sclerosis. Diffusion tensor imaging region of interest analysis of baseline scans showed differences between final diagnosis of multiple sclerosis and acute disseminated encephalomyelitis patients, whereby low fractional anisotropy and high radial diffusivity occurred in multiple sclerosis patients compared with acute disseminated encephalomyelitis patients and the age-matched controls. Fractional anisotropy and radial diffusivity measures may have the potential to serve as biomarkers for distinguishing acute disseminated encephalomyelitis from multiple sclerosis at the onset. Copyright © 2017 Elsevier Inc. All

  15. Neuromuscular ultrasound in polyneuropathies and motor neuron disease.

    Science.gov (United States)

    Hobson-Webb, Lisa D

    2013-06-01

    Current standards for diagnosing polyneuropathies (PN) and motor neuron disease (MND) sometimes lack early sensitivity and result in delayed diagnosis and treatment. Neuromuscular ultrasound (NMUS), already established in the diagnosis of entrapment neuropathies, may offer another means of diagnosis and monitoring response to therapy. This review of current evidence discusses diffuse nerve hypertrophy in hereditary demyelinating neuropathies, multifocal nerve enlargement in acquired demyelinating PN and the lack of readily apparent structural change in axonal neuropathies. NMUS detection of fasciculations and muscular change in MND is also reviewed, along with the need for further research to better define the role of nerve imaging in patients with PN. Copyright © 2013 Wiley Periodicals, Inc.

  16. A new pattern of spinal-cord demyelination in guinea pigs with acute experimental allergic encephalomyelitis mimicking multiple sclerosis.

    OpenAIRE

    Colover, J.

    1980-01-01

    A technique is described for producing large demyelinating lesions of the spinal cord in the guinea pig. Guinea pigs were pretreated by immunization with ovalbumin and water-soluble adjuvant (N-acetyl-muramyl L-alanyl D-isoglutamine, MDP) in water-in-oil emulsion (Freund's incomplete adjuvant). They were given a large dose (10 mg) of ovalbumin i.p. one month later. After a few weeks the animals were sensitized with guinea-pig basic protein in Freund's complete adjuvant. Five out of 11 animals...

  17. Treatment with metallothionein prevents demyelination and axonal damage and increases oligodendrocyte precursors and tissue repair during experimental autoimmune encephalomyelitis

    DEFF Research Database (Denmark)

    Penkowa, Milena; Hidalgo, Juan

    2003-01-01

    and neuroprotective proteins that are expressed during EAE and MS. We have shown recently that exogenous administration of Zn-MT-II to Lewis rats with EAE significantly reduced clinical symptoms and the inflammatory response, oxidative stress, and apoptosis of the infiltrated central nervous system areas. We show...... for the first time that Zn-MT-II treatment during EAE significantly prevents demyelination and axonal damage and transection, and stimulates oligodendroglial regeneration from precursor cells, as well as the expression of the growth factors basic fibroblast growth factor (bFGF), transforming growth factor (TGF...

  18. Actualización en el tratamiento de la neuropatía óptica inflamatoria desmielinizante Updating on the treatment of the demyelinating inflammatory optical neuropathy

    Directory of Open Access Journals (Sweden)

    Yaimara Hernández Silva

    2011-06-01

    Full Text Available Se realizó una revisión bibliográfica con el objetivo de proporcionar una actualización de las drogas que se emplean para retrasar la aparición de esclerosis múltiple en el manejo de la neuropatía óptica inflamatoria desmielinizante. El artículo presenta el origen y la justificación de la terapia esteroidea en este grupo de enfermedad, así como los mecanismos de acción y beneficios de tratamientos más modernos como los inmunomoduladores e inmunosupresores. El trabajo también introduce muchas de las drogas con efectos neuroprotectores que se encuentran en fases experimentales, cuyo uso prevendría la neurodegeneración que se produce a nivel de las células ganglionares retinianas en esta enfermedad neurológica. Las opciones terapéuticas actuales ofrecen variantes de tratamiento adicionales a pacientes con mayores probabilidades de desarrollo de esclerosis múltiple y retrasan la aparición de un segundo brote, así como las secuelas invalidantes que esta suele originar.A bibliographic review was conducted to provide an updating of drugs used to retard the appearance of multiple sclerosis in the management of the demyelinating inflammatory optical neuropathy. Present paper shows the origin and the justification of the steroid therapy in this disease, as well as the mechanisms of action and benefits of more recent treatments, e.g. the ongoing immunomodulations and immunosuppressive ones and also to introduce many drugs in experimental phase having neuroprotection effects whose use will prevent the neurodegenerative effect produced at level of the retinal ganglion cells in this neurologic disease. The current therapeutical options offer variants of additional treatment to those patients with greater possibilities to development multiple sclerosis and retarding the appearance of a second outbreak, as well as its disabling sequelae.

  19. Sildenafil (Viagra Protective Effects on Neuroinflammation: The Role of iNOS/NO System in an Inflammatory Demyelination Model

    Directory of Open Access Journals (Sweden)

    Catarina Raposo

    2013-01-01

    Full Text Available We recently demonstrated that sildenafil reduces the expression of cytokines, COX-2, and GFAP in a demyelinating model induced in wild-type (WT mice. Herein, the understandings of the neuroprotective effect of sildenafil and the mediation of iNOS/NO system on inflammatory demyelination induced by cuprizone were investigated. The cerebella of iNOS−/− mice were examined after four weeks of treatment with cuprizone alone or combined with sildenafil. Cuprizone increased GFAP, Iba-1, TNF-α, COX-2, IL-1β, and IFN-γ expression, decreased expression of glutathione S-transferase pi (GSTpi, and damaged myelin in iNOS−/− mice. Sildenafil reduced Iba-1, IFN-γ, and IL-1β levels but had no effect on the expression of GFAP, TNF-α, and COX-2 compared to the cuprizone group. Sildenafil elevated GSTpi levels and improved the myelin structure/ultrastructure. iNOS−/− mice suffered from severe inflammation following treatment with cuprizone, while WT mice had milder inflammation, as found in the previous study. It is possible that inflammatory regulation through iNOS-feedback is absent in iNOS−/− mice, making them more susceptible to inflammation. Sildenafil has at least a partial anti-inflammatory effect through iNOS inhibition, as its effect on iNOS−/− mice was limited. Further studies are required to explain the underlying mechanism of the sildenafil effects.

  20. Human induced pluripotent stem cells differentiation into oligodendrocyte progenitors and transplantation in a rat model of optic chiasm demyelination.

    Directory of Open Access Journals (Sweden)

    Alireza Pouya

    Full Text Available BACKGROUND: This study aims to differentiate human induced pluripotent stem cells (hiPSCs into oligodendrocyte precursors and assess their recovery potential in a demyelinated optic chiasm model in rats. METHODOLOGY/PRINCIPAL FINDINGS: We generated a cell population of oligodendrocyte progenitors from hiPSCs by using embryoid body formation in a defined medium supplemented with a combination of factors, positive selection and mechanical enrichment. Real-time polymerase chain reaction and immunofluorescence analyses showed that stage-specific markers, Olig2, Sox10, NG2, PDGFRα, O4, A2B5, GalC, and MBP were expressed following the differentiation procedure, and enrichment of the oligodendrocyte lineage. These results are comparable with the expression of stage-specific markers in human embryonic stem cell-derived oligodendrocyte lineage cells. Transplantation of hiPSC-derived oligodendrocyte progenitors into the lysolecithin-induced demyelinated optic chiasm of the rat model resulted in recovery from symptoms, and integration and differentiation into oligodendrocytes were detected by immunohistofluorescence staining against PLP and MBP, and measurements of the visual evoked potentials. CONCLUSIONS/SIGNIFICANCE: These results showed that oligodendrocyte progenitors generated efficiently from hiPSCs can be used in future biomedical studies once safety issues have been overcome.

  1. [Correlation between dental pulp demyelination degree and pain visual analogue scale scores data under acute and chronic pulpitis].

    Science.gov (United States)

    Korsantiia, N B; Davarashvili, X T; Gogiashvili, L E; Mamaladze, M T; Tsagareli, Z G; Melikadze, E B

    2013-05-01

    The aim of study is the analysis of pulp nerve fibers demyelination degree and its relationship with Visual Analogue Scale (VAS) score that may be measured as objective criteria. Material and methods of study. Step I: electron micrografs of dental pulp simples with special interest of myelin structural changes detected in 3 scores system, obtained from 80 patients, displays in 4 groups: 1) acute and 2) chronic pulpitis without and with accompined systemic deseases, 20 patients in each group. Dental care was realized in Kutaisi N1 Dental clinic. Step II - self-reported VAS used for describing dental pain. All data were performed by SPSS 10,0 version statistics including Spearmen-rank and Mann-Whitny coefficients for examine the validity between pulp demyelination degree and pain intensity in verbal, numbered and box scales. Researched Data were shown that damaged myelin as focal decomposition of membranes and Schwann cells hyperthrophia correspond with acute dental pain intensity as Spearman index reported in VAS numbered Scales, myelin and axoplasm degeneration as part of chronic gangrenous pulpitis disorders are in direct correlation with VAS in verbal, numbered and behavioral Rating Scales. In fact, all morphological and subjective data, including psychomotoric assessment of dental painin pulpitis may be used in dental practice for evaluation of pain syndrome considered personal story.

  2. Panencefalite subaguda esclerosante: participação da oligodendróglia no processo desmielinizante Subacute sclerosing panencephalitis (SSPE: the role of oligodendroglial cells in the demyelinating process

    Directory of Open Access Journals (Sweden)

    Alexandre Alberto de Alencar

    1973-01-01

    Full Text Available Estudo anátomo-clínico de 6 casos de panencefalite subaguda esclerosante (SSPE, onde se procura demonstrar serem as desmielinizações observadas nesta doença, na substância branca cerebral, causadas pela destruição, por parasitismo viral, das células oligodêndricas, que são as células responsáveis pela mielinização das fibras nervosas ao nível do sistema nervoso central. Após uma introdução em que se mostra, do ponto de vista histórico, a evolução dos conceitos histológicos a respeito da célula oligodêndrica, faz-se a apresentação anátomo-clínica dos 6 casos. Nos capítulos seguintes procura-se demonstrar que as alterações da mielina encontradas são devidas a destruição da oligodendróglia.It was made a clinical-pathological study of six (6 cases of subacute sclerosing panencephalitis (SSPE in order to demonstrate that the demyelinating process observed in this disease has its origin in the destruction of oligodendroglial cells by viral parasitism. As we know oligodendroglial cells are responsible for the myelination in the Central Nervous System. It was made a brief historical revision of the oligodendroglial cells concept and, after the description of the six cases, we tried to demonstrate that the demyelinating process observed are the result of oligodendroglial cells destruction.

  3. Papillary thyroid carcinoma metastasis to the lumbar spine masquerading as a schmorl's node

    Energy Technology Data Exchange (ETDEWEB)

    Daignault, Cory P.; Palmer, Edwin L.; Scott, James A.; Swan, John S. [Dept. of Radiology, Massachusetts General Hospital, Boston (United States); Daniels, Gilbert H. [Dept. of Radiology, Division of Nuclear Medicine, UMass Memorial Medical Center, Worcester (United States)

    2015-09-15

    A Schmorl's node is a common incidental finding encountered during radiologic imaging. Despite the vertebral body being a common site of metastatic disease, a lytic lesion adjacent to an endplate with typical imaging features can often confidently be called a Schmorl's node. This is a case report of a patient with a single well-defined FDG-avid papillary thyroid carcinoma metastasis to the spine that had imaging findings characteristic of a Schmorl's node on CT and MRI. This case is important to consider as it demonstrates that the imaging characteristics of metastatic disease and Schmorl's nodes can overlap.

  4. Pulsed high-dose dexamethasone versus standard prednisolone treatment for chronic inflammatory demyelinating polyradiculoneuropathy (PREDICT study): a double-blind, randomised, controlled trial

    NARCIS (Netherlands)

    van Schaik, Ivo N.; Eftimov, Filip; van Doorn, Pieter A.; Brusse, Esther; van den Berg, Leonard H.; van der Pol, W. Ludo; Faber, Catharina G.; van Oostrom, Joost Ch; Vogels, Oscar Jm; Hadden, Rob Dm; Kleine, Bert U.; van Norden, Anouk Gw; Verschuuren, Jan Jgm; Dijkgraaf, Marcel Gw; Vermeulen, Marinus

    2010-01-01

    Background Pulsed high-dose dexamethasone induced long-lasting remission in patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) in a pilot study. The PREDICT study aimed to compare remission rates in patients with CIDP treated with high-dose dexamethasone with rates in

  5. Subcutaneous immunoglobulin as first-line therapy in treatment-naive patients with chronic inflammatory demyelinating polyneuropathy

    DEFF Research Database (Denmark)

    Markvardsen, L. H.; Sindrup, S. H.; Christiansen, I.

    2017-01-01

    Background and purpose: Subcutaneous immunoglobulin (SCIG) is effective as maintenance treatment in chronic inflammatory demyelinating polyneuropathy (CIDP). We investigated whether multiple subcutaneous infusions are as effective as conventional therapy with intravenous loading doses in treatment...... treatment arm and followed for a further 10 weeks. All participants were evaluated at weeks 0, 2, 5 and 10 during both therapies. Primary outcome was combined isokinetic muscle strength (cIKS). Secondary outcomes were disability, clinical evaluation of muscle strength and the performance of various function...... tests. Results: All participants received both therapies, 14 completing the protocol. Overall, cIKS increased by 7.4 ± 14.5% (P = 0.0003) during SCIG and by 6.9 ± 16.8% (P = 0.002) during IVIG, the effect being similar (P = 0.80). Improvement of cIKS peaked 2 weeks after IVIG and 5 weeks after SCIG...

  6. Large Omental Cystic Lymphangioma Masquerading as Mucinous Ovarian Neoplasia in an 8-Year-Old Premenarchal Girl: The Findings from Diagnostic Imaging and Laparoscopic-Assisted Excision.

    Science.gov (United States)

    Takeda, Akihiro; Ito, Hiroaki; Nakamura, Hiromi

    2017-12-01

    Omental cystic lymphangioma is an extremely rare abdominal mass caused by congenital malformation. An 8-year-old premenarchal girl reported abdominal pain. Diagnostic imaging revealed a large multicystic mass measuring 22 cm in diameter, which occupied the entire abdominal cavity with ascites. Emergency laparoscopy revealed a ruptured large cystic mass originating from the greater omentum; this was followed by successful laparoscopic-assisted excision. The pathological diagnosis was omental cystic lymphangioma. The present findings show that omental cystic lymphangioma masquerading as mucinous ovarian neoplasia was a rare cause of acute abdominal events in a young girl. The present case shows that minimally invasive surgery can be a feasible option, which might achieve a favorable outcome in a young patient with an omental cystic lymphangioma. Copyright © 2017 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. All rights reserved.

  7. A molar masquerading as an ectopic pregnancy in the early first trimester: a salutary lesson

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    Govind A

    2012-08-01

    Full Text Available Abha Govind,1 Nisha Lakhi21Department of Obstetrics and Gynaecology, North Middlesex University Hospital, London, UK; 2Department of Obstetrics and Gynecology, Richmond University Medical Center, Staten Island, New York, NY, USAAbstract: The authors report a case in which a molar pregnancy was mistaken for an ectopic pregnancy in the early first trimester. This confusion delayed diagnosis and caused distress; follow-up led to the final diagnosis of complete hydatidiform mole. Correct preoperative diagnosis of molar pregnancy by ultrasound in early pregnancy may be tricky. It is important to obtain histological evidence to make the final diagnosis of gestational trophoblastic disease. While relatively rare, consideration of molar pregnancy in the differential diagnosis of early pregnancy loss may avoid unnecessary distress.Keywords: first trimester ultrasound, molar pregnancy, gestational trophoblastic disease, complete hydatidiform mole, diagnosis

  8. IgG4-related intraocular inflammation masquerading as ciliary body melanoma in a young girl

    Directory of Open Access Journals (Sweden)

    Dipankar Das

    2016-01-01

    Full Text Available Immunoglobulin G4 (IgG4-related diseases affects various tissues and organs of the human body. Orbital, adnexal, and scleral inflammations were already reported in the medical literature. To the best of our knowledge, we report the first case of intraocular IgG4-associated inflammatory mass in the ciliary body mimicking as a melanoma in a 23-year-old female from Northeast India. Characteristic histopathology, immunohistochemistry in the tissue, protein chemistry, and raised serum IgG4 were supportive for the diagnosis. As this newly diagnosed disease has multi-organ affection and little is known about its pathogenesis particularly in eye and adnexa, the present case will open many challenges in clinico-pathological diagnosis and research in the future.

  9. Retroperitoneal Mass Masquerading as Failure to Thrive in a 91-year-old Woman.

    Science.gov (United States)

    Ologun, Gabriel O; Yarze, Noel; Bertsch, David; Mwesige, Joseph

    2017-11-08

    Failure to thrive (FTT) is a state of overall decline. Patients often present with weight loss, poor appetite, malnutrition, and decreased physical functioning. The etiology is multifactorial including chronic diseases, functional impairments, and acute illnesses. Evaluation for reversible causes is paramount, and treatment is aimed at maintaining or improving functional status. We present a case of a 91-year-old woman with a retroperitoneal mass that was found on workup for failure to thrive.

  10. Primary cutaneous anaplastic large cell lymphoma masquerading as large pyogenic granuloma

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    Anupama Bains

    2016-01-01

    Full Text Available Primary cutaneous anaplastic large cell lymphoma (pcALCL forms 9% of the cutaneous T-cell lymphomas. It usually presents as solitary reddish brown ulcerating nodule or indurated plaque. Sometimes, it mimics other dermatological diseases such as eczema, pyoderma gangrenosum, pyogenic granuloma, morphea, and squamous cell carcinoma. Our case presented with large pyogenic granuloma like lesion with regional lymphadenopathy. Since pcALCL is rare, one can misdiagnose such cases and therefore high index of suspicion is necessary.

  11. A case of mistaken identity: When lupus masquerades as primary myelofibrosis

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    Edy Hasrouni

    2013-08-01

    Full Text Available Introduction: Autoimmune myelofibrosis is an uncommon hematologic disease characterized by anemia, bone marrow myelofibrosis, and an autoimmune feature. Myelofibrosis is often associated with other conditions, including infections, nutritional/endocrine dysfunction, toxin/drug exposure, and connective tissue diseases, including scleroderma and systemic lupus erythematosus. Absence of clonal markers ( JAK2 and heterogeneity of the symptoms often complicate the diagnosis. Case presentation: Here, we present two cases of systemic lupus erythematosus–induced autoimmune myelofibrosis. The first case is of a 36-year-old African American female with diagnosis of systemic lupus erythematosus at the age of 12 years. The second patient is a 44-year-old African American male with family history of systemic lupus erythematosus who developed anemia and constitutional symptoms later on. Both patients showed hypercellularity and fibrotic changes of the bone marrow. Moreover, mutational analysis showed that both patients were wild type for JAK2 (V617F and exon 12 and MPL (exon 10. Conclusions: These two cases illustrate that anemic patients with fibrotic changes in the bone marrow without other clinicopathologic features associated with primary myelofibrosis in the presence of clinical manifestations and history of an autoimmune disease should suggest an autoimmune myelofibrosis. These cases demonstrate that a good clinical history combined with molecular technologies and pathomorphologic criteria are helpful in distinguishing between primary myelofibrosis and a nonclonal myelofibrosis from an associated condition.

  12. Rhinomaxillary mucormycosis masquerading as chronic osteomyelitis: A series of four rare cases with review of literature

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    Neeta Mohanty

    2012-01-01

    Full Text Available In a dental hospital, patients reporting with exposed bone in the palate/maxilla with or without pus discharging sinuses are generally clinically diagnosed as osteomyelitis which can occur as a complication of odontogenic bacterial infections, traumatic injuries, herpes zoster infection, aspergillosis, mucormycosis or iatrogenic infections. We present a series of four cases, all of which were initially clinically diagnosed as osteomyelitis and later confirmed to be mucormycosis following histopathological examination. Although rare, the common form of this opportunistic fungal infection is seen in the rhinomaxillary region and in people with an underlying systemic disease like diabetes mellitus (DM. This case series of rhinomaxillary mucormycosis is being reported to increase awareness among dental surgeons to regard the occurrence of osteomyelitis in the maxilla occurring in a immunocompromised patient especially with poorly controlled DM, with suspicion of an aggressive, fulminant, fatal fungal infection so as to ensure an early diagnosis and prompt treatment thereby reducing the morbidity and mortality associated with this disease.

  13. Utility of CSF Cytokine/Chemokines as Markers of Active Intrathecal Inflammation: Comparison of Demyelinating, Anti-NMDAR and Enteroviral Encephalitis.

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    Kavitha Kothur

    Full Text Available Despite the discovery of CSF and serum diagnostic autoantibodies in autoimmune encephalitis, there are still very limited CSF biomarkers for diagnostic and monitoring purposes in children with inflammatory or autoimmune brain disease. The cause of encephalitis is unknown in up to a third of encephalitis cohorts, and it is important to differentiate infective from autoimmune encephalitis given the therapeutic implications.To study CSF cytokines and chemokines as diagnostic biomarkers of active neuroinflammation, and assess their role in differentiating demyelinating, autoimmune, and viral encephalitis.We measured and compared 32 cytokine/chemokines using multiplex immunoassay and APRIL and BAFF using ELISA in CSF collected prior to commencing treatment from paediatric patients with confirmed acute disseminated encephalomyelitis (ADEM, n = 16, anti-NMDAR encephalitis (anti-NMDAR E, n = 11, and enteroviral encephalitis (EVE, n = 16. We generated normative data using CSF from 20 non-inflammatory neurological controls. The sensitivity of CSF cytokine/chemokines to diagnose encephalitis cases was calculated using 95th centile of control values as cut off. We correlated CSF cytokine/chemokines with disease severity and follow up outcome based on modified Rankin scale. One-way hierarchical correlational cluster analysis of molecules was performed in different encephalitis and outcome groups.In descending order, CSF TNF-α, IL-10, IFN-α, IL-6, CXCL13 and CXCL10 had the best sensitivity (>79.1% when all encephalitis patients were included. The combination of IL-6 and IFN-α was most predictive of inflammation on multiple logistic regression with area under the ROC curve 0.99 (CI 0.97-1.00. There were no differences in CSF cytokine concentrations between EVE and anti-NMDAR E, whereas ADEM showed more pronounced elevation of Th17 related (IL-17, IL-21 and Th2 (IL-4, CCL17 related cytokine/chemokines. Unlike EVE, heat map analysis showed similar clustering

  14. Prostatic Lymphoma Masquerading as Urinary Retention and Hematuria With Review of Literature

    Science.gov (United States)

    Tamang, Tsering Gyalpo Lama; Singh, Prabhsimranjot; Garellek, Jonathan; Malhotra, Sonali; Chandra, Abhinav Binod; Solomon, William

    2017-01-01

    Lymphomas of prostate are very rare tumors. They are not commonly considered in the clinical and histological differential diagnosis of prostatic enlargement. We report a case of a 49-year-old man who presented to emergency department with several weeks of difficulty in urination, for which he was being treated for benign prostate hyperplasia with no improvement. Computerized tomography scan showed lobulated mass originating from the superior aspect of the prostate with right inguinal lymph node involvement and no distant organ metastatic disease. Prostatic biopsy revealed diffuse large B-cell lymphoma. The patient achieved complete remission after six cycles of rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone chemotherapy regimen. Lymphomas of the prostate should be considered in differential diagnosis of the patient presenting with obstructive lower urinary tract symptoms especially in patients with normal prostatic-specific antigen level and previous history of lymphoma in other sites. PMID:29147449

  15. Posterior reversible encephalopathy syndrome masquerading as progressive multifocal leukoencephalopathy in rituximab treated neuromyelitis optica.

    Science.gov (United States)

    Berger, Joseph R; Neltner, Janna; Smith, Charles; Cambi, Franca

    2014-11-01

    Both progressive multifocal leukoencephalopathy (PML) and posterior reversible encephalopathy syndrome (PRES) have been reported as complications of rituximab therapy. These disorders may appear indistinguishable on magnetic resonance imaging (MRI). We report on a 42 year old woman with neuromyelitis optica (NMO) of 10 years duration who developed extensive white matter disease affecting chiefly both parietal lobes 6 months after her first and only dose of rituximab. The MRI findings suggested the diagnosis of PML, but her history was more consistent with PRES. Ultimately, a brain biopsy was performed which was consistent with the diagnosis of PRES. PRES and PML may have overlapping symptomatology and be indistinguishable on MRI. An approach to distinguishing between these two disorders is addressed. Copyright © 2014. Published by Elsevier B.V.

  16. Deficient Surveillance and Phagocytic Activity of Myeloid Cells Within Demyelinated Lesions in Aging Mice Visualized byEx VivoLive Multiphoton Imaging.

    Science.gov (United States)

    Rawji, Khalil S; Kappen, Janson; Tang, Weiwen; Teo, Wulin; Plemel, Jason R; Stys, Peter K; Yong, V Wee

    2018-02-21

    Aging impairs regenerative processes including remyelination, the synthesis of a new myelin sheath. Microglia and other infiltrating myeloid cells such as macrophages are essential for remyelination through mechanisms that include the clearance of inhibitory molecules within the lesion. Prior studies have shown that the quantity of myeloid cells and the clearance of inhibitory myelin debris are deficient in aging, contributing to the decline in remyelination efficiency with senescence. It is unknown, however, whether the impaired clearance of debris is simply the result of the reduced number of phagocytes or if the dynamic activity of myeloid cells within the demyelinating plaque also declines with aging and this question is relevant to the proper design of therapeutics to mobilize myeloid cells for repair. Herein, we describe a high-resolution multiphoton ex vivo live imaging protocol that visualizes individual myelinated/demyelinated axons and lipid-containing myeloid cells to investigate the demyelinated lesion of aging female mice. We found that aging lesions have fewer myeloid cells and that these have reduced phagocytosis of myelin. Although the myeloid cells are actively migratory within the lesion of young mice and have protrusions that seem to survey the environment, this motility and surveillance is significantly reduced in aging mice. Our results emphasize the necessity of not only increasing the number of phagocytes, but also enhancing their activity once they are within demyelinated lesions. The high-resolution live imaging of demyelinated lesions can serve as a platform with which to discover pharmacological agents that rejuvenate intralesional remodeling that promotes the repair of plaques. SIGNIFICANCE STATEMENT The repair of myelin after injury depends on myeloid cells that clear debris and release growth factors. As organisms age, remyelination becomes less efficient correspondent with fewer myeloid cells that populate the lesions. It is unknown

  17. Effects of the PPAR-β agonist GW501516 in an in vitro model of brain inflammation and antibody-induced demyelination

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    Honegger Paul

    2009-05-01

    Full Text Available Abstract Background Brain inflammation plays a central role in numerous brain pathologies, including multiple sclerosis (MS. Microglial cells and astrocytes are the effector cells of neuroinflammation. They can be activated also by agents such as interferon-γ (IFN-γ and lipopolysaccharide (LPS. Peroxisome proliferator-associated receptor (PPAR pathways are involved in the control of the inflammatory processes, and PPAR-β seems to play an important role in the regulation of central inflammation. In addition, PPAR-β agonists were shown to have trophic effects on oligodendrocytes in vitro, and to confer partial protection in experimental autoimmune encephalomyelitis (EAE, an animal model of MS. In the present work, a three-dimensional brain cell culture system was used as in vitro model to study antibody-induced demyelination and inflammatory responses. GW 501516, a specific PPAR-β agonist, was examined for its capacity to protect from antibody-mediated demyelination and to prevent inflammatory responses induced by IFN-γ and LPS. Methods Aggregating brain cells cultures were prepared from embryonal rat brain, and used to study the inflammatory responses triggered by IFN-γ and LPS and by antibody-mediated demyelination induced by antibodies directed against myelin-oligodendrocyte glycoprotein (MOG. The effects of GW 501516 on cellular responses were characterized by the quantification of the mRNA expression of tumor necrosis factor-α (TNF-α, interleukin-6 (IL-6, inducible NO synthase (i-NOS, PPAR-β, PPAR-γ, glial fibrillary acidic protein (GFAP, myelin basic protein (MBP, and high molecular weight neurofilament protein (NF-H. GFAP expression was also examined by immunocytochemistry, and microglial cells were visualized by isolectin B4 (IB4 and ED1 labeling. Results GW 501516 decreased the IFN-γ-induced up-regulation of TNF-α and iNOS in accord with the proposed anti-inflammatory effects of this PPAR-β agonist. However, it increased IL

  18. Hodgkin's lymphoma masquerading as vertebral osteomyelitis in a man with diabetes: a case report

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    Sohani Aliyah R

    2010-04-01

    Full Text Available Abstract Introduction Infection and malignancy often have common characteristics which render the differential diagnosis for a prolonged fever difficult. Imaging and tissue biopsy are crucial in making a correct diagnosis, though differentiating between chronic osteomyelitis and malignancy is not always straightforward as they possess many overlapping features. Case Presentation A 52-year-old Caucasian man was treated with antibiotics for his diabetic foot infection after a superficial culture showed Staphylococcus aureus. He had persistent fevers for several weeks and later developed acute onset of back pain which was treated with several courses of antibiotics. Radiographic and pathological findings were atypical, and a diagnosis of Hodgkin's lymphoma was made 12 weeks later. Conclusion Clinicians should maintain a suspicion for Hodgkin's lymphoma or other occult malignancy when features of presumed osteomyelitis are atypical. Chronic vertebral osteomyelitis in particular often lacks features common to acute infectious disease processes, and the chronic lymphocytic infiltrates seen on histopathology have very similar features to Hodgkin's lymphoma, highlighting a similar inflammatory microenvironment sustained by both processes.

  19. Hand involvement in children with Charcot-Marie-Tooth disease type 1A

    NARCIS (Netherlands)

    Burns, Joshua; Bray, Paula; Cross, Lauren A.; North, Kathryn N.; Ryan, Monique M.; Ouvrier, Robert A.

    2008-01-01

    Charcot-Marie-Tooth disease type 1A (CMT1A), a demyelinating neuropathy characterised by progressive length-dependent muscle weakness and atrophy, is thought to affect the foot and leg first followed some time later by hand weakness and dysfunction. We aimed to characterise hand Strength, function

  20. European Federation of Neurological Societies/Peripheral Nerve Society guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy: report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society

    NARCIS (Netherlands)

    Hughes, R. A. C.; Bouche, P.; Cornblath, D. R.; Evers, E.; Hadden, R. D. M.; Hahn, A.; Illa, I.; Koski, C. L.; Léger, J. M.; Nobile-Orazio, E.; Pollard, J.; Sommer, C.; van den Bergh, P.; van Doorn, P. A.; van Schaik, I. N.

    2006-01-01

    Numerous sets of diagnostic criteria have sought to define chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and randomized trials and systematic reviews of treatment have been published. The objective is to prepare consensus guidelines on the definition, investigation and treatment

  1. Transfusion-transmitted malaria masquerading as sickle cell crisis with multisystem organ failure.

    Science.gov (United States)

    Maier, Cheryl L; Gross, Phillip J; Dean, Christina L; Chonat, Satheesh; Ip, Andrew; McLemore, Morgan; El Rassi, Fuad; Stowell, Sean R; Josephson, Cassandra D; Fasano, Ross M

    2018-03-09

    Fever accompanying vaso-occlusive crisis is a common presentation in patients with sickle cell disease (SCD) and carries a broad differential diagnosis. Here, we report a case of transfusion-transmitted malaria in a patient with SCD presenting with acute vaso-occlusive crisis and rapidly decompensating to multisystem organ failure (MSOF). An 18-year-old African American male with SCD was admitted after multiple days of fever and severe generalized body pain. He received monthly blood transfusions as stroke prophylaxis. A source of infection was not readily identified, but treatment was initiated with continuous intravenous fluids and empiric antibiotics. The patient developed acute renal failure, acute hypoxic respiratory failure, and shock. He underwent red blood cell (RBC) exchange transfusion followed by therapeutic plasma exchange and continuous veno-venous hemodialysis. A manual peripheral blood smear revealed intraerythrocytic inclusions suggestive of Plasmodium, and molecular studies confirmed Plasmodium falciparum infection. Intravenous artesunate was given daily for 1 week. A look-back investigation involving two hospitals, multiple blood suppliers, and state and federal public health departments identified the source of malaria as a unit of RBCs transfused 2 weeks prior to admission. Clinical suspicion for transfusion-related adverse events, including hemolytic transfusion reactions and transfusion-transmitted infections, should be high in typically and atypically immunocompromised patient populations (like SCD), especially those on chronic transfusion protocols. Manual blood smear review aids in the evaluation of patients with SCD presenting with severe vaso-occlusive crisis and MSOF and can alert clinicians to the need for initiating aggressive therapy like RBC exchange and artesunate therapy. © 2018 AABB.

  2. Increased demyelination and axonal damage in metallothionein I+II-deficient mice during experimental autoimmune encephalomyelitis

    DEFF Research Database (Denmark)

    Penkowa, M; Espejo, C; Martínez-Cáceres, E M

    2003-01-01

    Metallothioneins I+II (MT-I+II) are antioxidant, neuroprotective factors. We previously showed that MT-I+II deficiency during experimental autoimmune encephalomyelitis (EAE) leads to increased disease incidence and clinical symptoms. Moreover, the inflammatory response of macrophages and T cells...

  3. Pediatric-onset multiple sclerosis and other acquired demyelinating syndromes of the central nervous system in Denmark during 1977-2015

    DEFF Research Database (Denmark)

    Boesen, Magnus Spangsberg; Magyari, Melinda; Koch-Henriksen, Nils

    2018-01-01

    BACKGROUND: The incidence of acquired demyelinating syndromes (ADS) including multiple sclerosis (MS) has never been investigated in a Danish pediatric population. OBJECTIVES: We estimated the nationwide age- and sex-specific incidence of pediatric ADS including MS. METHODS: Data were sourced from...... European countries. Referral bias and classification differences may account for this disparity, in particular the age-intervals and the definition of onset....

  4. Progesterone therapy induces an M1 to M2 switch in microglia phenotype and suppresses NLRP3 inflammasome in a cuprizone-induced demyelination mouse model.

    Science.gov (United States)

    Aryanpour, Roya; Pasbakhsh, Parichehr; Zibara, Kazem; Namjoo, Zeinab; Beigi Boroujeni, Fatemeh; Shahbeigi, Saeed; Kashani, Iraj Ragerdi; Beyer, Cordian; Zendehdel, Adib

    2017-10-01

    Demyelination of the central nervous system (CNS) has been associated to reactive microglia in neurodegenerative disorders, such as multiple sclerosis (MS). The M1 microglia phenotype plays a pro-inflammatory role while M2 is involved in anti-inflammatory processes in the brain. In this study, CPZ-induced demyelination mouse model was used to investigate the effect of progesterone (PRO) therapy on microglia activation and neuro-inflammation. Results showed that progesterone therapy (CPZ+PRO) decreased neurological behavioral deficits, as demonstrated by significantly decreased escape latencies, in comparison to CPZ mice. In addition, CPZ+PRO caused a significant reduction in the mRNA expression levels of M1-markers (iNOS, CD86, MHC-II and TNF-α) in the corpus callosum region, whereas the expression of M2-markers (Trem-2, CD206, Arg-1 and TGF-β) was significantly increased, in comparison to CPZ mice. Moreover, CPZ+PRO resulted in a significant decrease in the number of iNOS + and Iba-1 + /iNOS + cells (M1), whereas TREM-2 + and Iba-1 + /TREM-2 + cells (M2) significantly increased, in comparison to CPZ group. Furthermore, CPZ+PRO caused a significant decrease in mRNA and protein expression levels of NLRP3 and IL-18 (~2-fold), in comparison to the CPZ group. Finally, CPZ+PRO therapy was accompanied with reduced levels of demyelination, compared to CPZ, as confirmed by immunofluorescence to myelin basic protein (MBP) and Luxol Fast Blue (LFB) staining, as well as transmission electron microscopy (TEM) analysis. In summary, we reported for the first time that PRO therapy causes polarization of M2 microglia, attenuation of M1 phenotype, and suppression of NLRP3 inflammasome in a CPZ-induced demyelination model of MS. Copyright © 2017 Elsevier B.V. All rights reserved.

  5. Application of Mesenchymal Stem Cell Intended to Investigate the Reward Response of Nucleus Accumbens in a Local Demyelination Model of Rat

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    M. Goodarzvand

    2014-05-01

    Full Text Available Introduction: Demyelination is the most common complication of multiple sclerosis in that the myelin sheaths around the axons and cerebral nucleus are damaged. The accumbens nucleus is a part of the striatum of the CNS that has role in reward response. Recently, stem cell therapy has been considered by many researchers. Therefore, the aim of this study was application of mesenchymal stem cells to assess demyelination (apoptosis, remyelination (neuronal differentiation and reward response of accumbens nucleus in a local demyelination model. Method and Materials: Male Wistar rats weighting 250-300 gr were anesthetized with chloral hydrate. Ethidium bromide was directly injected into the nucleus accumbens. A thin canola was mounted into the ventricle coordination, simultaneously. Animals were divided into 4 groups: Control (received saline as solvent of EB, sham, PBS 14 and 28 (received PBS as vehicle of stem cell and experiments 14 and 28 (received mesenchymal 5* 100000 of stem cells. Conditioned Place Preference (CPP was done in days zero, 14 and 28 post surgery and morphine was subcutaneously injected to assess reward response. Results: This study showed CCP increment in experiment groups compared to PBS ones, significantly. Discussion: The results of the present study suggesting stem cell therapy as an effective factor to swift the healing processes of the CNS and to improve reward response should be considered.

  6. Neurofascin (NF155- and NF186-Specific T Cell Response in a Patient Developing a Central Pontocerebellar Demyelination after 10 Years of CIDP

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    Juliane Klehmet

    2017-12-01

    Full Text Available BackgroundInformation and pathobiological understanding about central demyelinating manifestation in patients, who primarily suffer from chronic inflammatory demyelinating polyneuropathy (CIDP, are scarce.MethodsIFN-γ-response as well as antibodies against the (paranodal antigens neurofascin (NF155 and NF 186 had been tested by Elispot assay and ELISA before clinical manifestation and at follow-up.Case description and resultsThe patient described here developed a subacute brainstem syndrome more than 10 years after diagnosis of CIDP under low-dose maintenance treatment of intravenous immunoglobulins (IVIG. MRI revealed enhancing right-sided pontocerebellar lesion. CSF examination showed mild pleocytosis and elevated protein, and negative oligoclonal bands. Further diagnostics exclude differential diagnoses such as tuberculoma, sarcoidosis, or metastasis. Specific IFN-γ response against NF155 and NF186 as measured by Elispot assay was elevated before clinical manifestation. NF155 and NF186 antibodies were negative. Escalation of IVIG treatment at 2 g/kg BW followed by 1.4 g/kg BW led to clinical remission albeit to a new asymptomatic central lesion. Follow-up NF155 and NF186-Elispot turned negative.ConclusionThe case reported here with a delayed central manifestation after an initially typical CIDP and NF155 and NF186 T cell responses does not resemble described cases of combined central and peripheral demyelination but may reflect a novel subtype within the great clinical heterogeneity of CIDP.

  7. Promoting remyelination: utilizing a viral model of demyelination to assess cell-based therapies

    OpenAIRE

    Marro, Brett S; Blanc, Caroline A; Loring, Jeanne F; Cahalan, Michael D; Lane, Thomas E

    2014-01-01

    Multiple sclerosis (MS) is a chronic inflammatory disease of the CNS. While a broad range of therapeutics effectively reduce the incidence of focal white matter inflammation and plaque formation for patients with relapse-remitting forms of MS, a challenge within the field is to develop therapies that allow for axonal protection and remyelination. In the last decade, growing interest has focused on utilizing neural precursor cells (NPCs) to promote remyelination. To understand how NPCs functio...

  8. Subependymal Zone-Derived Oligodendroblasts Respond to Focal Demyelination but Fail to Generate Myelin in Young and Aged Mice

    Directory of Open Access Journals (Sweden)

    Ilias Kazanis

    2017-03-01

    Full Text Available Two populations of oligodendrogenic progenitors co-exist within the corpus callosum (CC of the adult mouse. Local, parenchymal oligodendrocyte progenitor cells (pOPCs and progenitors generated in the subependymal zone (SEZ cytogenic niche. pOPCs are committed perinatally and retain their numbers through self-renewing divisions, while SEZ-derived cells are relatively “young,” being constantly born from neural stem cells. We compared the behavior of these populations, labeling SEZ-derived cells using hGFAP:CreErt2 mice, within the homeostatic and regenerating CC of the young-adult and aging brain. We found that SEZ-derived oligodendroglial progenitors have limited self-renewing potential and are therefore not bona fide OPCs but rather “oligodendroblasts” more similar to the neuroblasts of the neurogenic output of the SEZ. In the aged CC their mitotic activity is much reduced, although they still act as a “fast-response element” to focal demyelination. In contrast to pOPCs, they fail to generate mature myelinating oligodendrocytes at all ages studied.

  9. Fluvoxamine stimulates oligodendrogenesis of cultured neural stem cells and attenuates inflammation and demyelination in an animal model of multiple sclerosis.

    Science.gov (United States)

    Ghareghani, Majid; Zibara, Kazem; Sadeghi, Heibatollah; Dokoohaki, Shima; Sadeghi, Hossein; Aryanpour, Roya; Ghanbari, Amir

    2017-07-07

    Multiple Sclerosis (MS) require medications controlling severity of the pathology and depression, affecting more than half of the patients. In this study, the effect of antidepressant drug fluvoxamine, a selective serotonin reuptake inhibitor, was investigated in vitro and in vivo. Nanomolar concentrations of fluvoxamine significantly increased cell viability and proliferation of neural stem cells (NSCs) through increasing mRNA expression of Notch1, Hes1 and Ki-67, and protein levels of NICD. Also, physiological concentrations of fluvoxamine were optimal for NSC differentiation toward oligodendrocytes, astrocytes and neurons. In addition, fluvoxamine attenuated experimental autoimmune encephalomyelitis (EAE) severity, a rat MS model, by significantly decreasing its clinical scores. Moreover, fluvoxamine treated EAE rats showed a decrease in IFN-γ serum levels and an increase in IL-4, pro- and anti-inflammatory cytokines respectively, compared to untreated EAE rats. Furthermore, immune cell infiltration and demyelination plaque significantly decreased in spinal cords of fluvoxamine-treated rats, which was accompanied by an increase in protein expression of MBP and GFAP positive cells and a decrease in lactate serum levels, a new biomarker of MS progression. In summary, besides its antidepressant activity, fluvoxamine stimulates proliferation and differentiation of NSCs particularly toward oligodendrocytes, a producer of CNS myelin.

  10. Oxidative stress in a model of toxic demyelination in rat brain: the effect of piracetam and vinpocetine.

    Science.gov (United States)

    Abdel-Salam, Omar M E; Khadrawy, Yasser A; Salem, Neveen A; Sleem, Amany A

    2011-06-01

    We studied the role of oxidative stress and the effect of vinpocetine (1.5, 3 or 6 mg/kg) and piracetam (150 or 300 mg/kg) in acute demyelination of the rat brain following intracerebral injection of ethidium bromide (10 μl of 0.1%). ethidium bromide caused (1) increased malondialdehyde (MDA) in cortex, hippocampus and striatum; (2) decreased total antioxidant capacity (TAC) in cortex, hippocampus and striatum; (3) decreased reduced glutathione (GSH) in cortex and hippocampus (4); increased serum nitric oxide and (5) increased striatal (but not cortical or hippocampal) acetylcholinesterase (AChE) activity. MDA decreased in striatum and cortex by the lower doses of vinpocetine or piracetam but increased in cortex and hippocampus and in cortex, hypothalamus and striatum by the higher dose of vinpocetine or piracetam, respectively along with decreased TAC. GSH increased by the higher dose of piracetam and by vinpocetine which also decreased serum nitric oxide. Vinpocetine and piracetam displayed variable effects on regional AChE activity.

  11. Multiple Sites Ultrasonography of Peripheral Nerves in Differentiating Charcot–Marie–Tooth Type 1A from Chronic Inflammatory Demyelinating Polyradiculoneuropathy

    Directory of Open Access Journals (Sweden)

    Jingwen Niu

    2017-05-01

    Full Text Available IntroductionMultiple sites measurement of cross-sectional areas (CSA by ultrasound was performed to differentiate Charcot–Marie–Tooth type 1A (CMT1A and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP.MethodsNine patients with CMT1A, 28 patients with CIDP, and 14 healthy controls (HC were recruited prospectively. Consecutive ultrasonography scanning was performed from wrist to axilla on median and ulnar nerves. CSAs were measured at 10 predetermined sites of each nerve.ResultsCMT1A had significantly larger CSAs at all sites of median and ulnar nerves (p < 0.01. In CMT1A, CSAs increased gradually and homogeneously from distal to proximal along the nerve, except potential entrapment sites. CIDP displayed three different morphological patterns, including mild enlargement in 15 patients, prominent segmental enlargement in 12, and slight enlargement in 1, among which different treatment responses were observed. All patients with mild nerve enlargement treated with intravenous immunoglobulin were responsive (7/7, while less than half of those with prominent segmental enlargement (3/7 were responsive (p < 0.01.DiscussionConsecutive scan along the nerve and multiple sites measurement by ultrasound could supply more detailed morphological feature of the nerve and help to differentiate CMT1A from CIDP.

  12. Japanese macaque encephalomyelitis: a spontaneous multiple sclerosis-like disease in a nonhuman primate

    Science.gov (United States)

    Axthelm, Michael K.; Bourdette, Dennis N.; Marracci, Gail H.; Su, Weiping; Mullaney, Elizabeth T.; Manoharan, Minsha; Kohama, Steven G.; Pollaro, Jim; Witkowski, Ellen; Wang, Paul; Rooney, William D.; Sherman, Lawrence S.; Wong, Scott W.

    2011-01-01

    Objective To describe Japanese macaque encephalomyelitis (JME), a spontaneous inflammatory demyelinating disease occurring in the Oregon National Primate Research Center’s (ONPRC) colony of Japanese macaques (JM, Macaca fuscata). Methods JM with neurologic impairment were removed from the colony, evaluated and treated with supportive care. Animals were humanely euthanized and their central nervous system (CNS) examined. Results ONPRC’s JM colony was established in 1965 and no cases of JME occurred until 1986. Since 1986, 56 JM spontaneously developed a disease characterized clinically by paresis of one or more limbs, ataxia or ocular motor paresis. Most animals were humanely euthanized during their initial episode. Three recovered, later relapsed and were then euthanized. There was no gender predilection and the median age for disease was 4 years. Magnetic resonance imaging of eight cases of JME revealed multiple gadolinium enhancing T1-weighted hyperintensities in the white matter of the cerebral hemispheres, brainstem, cerebellum and cervical spinal cord. The CNS of monkeys with JME contained multifocal plaque-like demyelinated lesions of varying ages, including acute and chronic, active demyelinating lesions with macrophages and lymphocytic periventricular infiltrates, and chronic, inactive demyelinated lesions. A previously undescribed gamma-herpesvirus was cultured from acute JME white matter lesions. Cases of JME continue to affect 1–3% of the ONPRC colony per year. Interpretation JME is a unique spontaneous disease in a nonhuman primate that has similarities with multiple sclerosis (MS) and is associated with a novel simian herpesvirus. Elucidating the pathogenesis of JME may shed new light on MS and other human demyelinating diseases. PMID:21674589

  13. Thyroid disease in older people.

    Science.gov (United States)

    Mitrou, Panayota; Raptis, Sotirios A; Dimitriadis, George

    2011-09-01

    Several changes in thyroid hormone secretion, metabolism, and action occur with the increase in age. Aging is often associated with a decrease in serum thyroid stimulating hormone and T3 levels, whereas serum free T4 levels usually remain unchanged. The prevalence of thyroid dysfunction is higher in the elderly as compared to the younger population. In elderly individuals the non-specific clinical manifestations of thyroid hormone excess or deprivation can cause confusion in the clinical setup; while some of the symptoms of thyroid disease are similar to those in younger patients, it is not uncommon for both hyperthyroidism and hypothyroidism to be manifested in subtle ways in older patients, often mimicking symptoms of aging or masquerading as diseases of the cardiovascular, gastrointestinal, or nervous system. In addition, diagnosis of thyroid disorders is commonly complicated, due to chronic, non-thyroidal illness or medication therapy. Early diagnosis and treatment of overt thyroid disorders is crucial, since these disorders are associated with increased morbidity and mortality in the elderly, usually due to common coexistent diseases such as diminished cardiovascular reserve. Treatment of subclinical thyroid disease should also be considered, based on a combination of age, symptoms and risk factors in the individual patients. In addition, both prevalence and aggressiveness of thyroid cancer increase with age. This review summarizes the changes of thyroid function, as well as the clinical manifestations and treatment of thyroid disorders with advancing age. Copyright © 2011 Elsevier Ireland Ltd. All rights reserved.

  14. Cytological artifacts masquerading interpretation

    Directory of Open Access Journals (Sweden)

    Khushboo Sahay

    2013-01-01

    Conclusions: In order to justify a cytosmear interpretation, a cytologist must be well acquainted with delayed fixation-induced cellular changes and microscopic appearances of common contaminants so as to implicate better prognosis and therapy.

  15. Genetic analysis of inflammation, cytokine mRNA expression and disease course of relapsing experimental autoimmune encephalomyelitis in DA rats

    DEFF Research Database (Denmark)

    Lorentzen, J C; Andersson, M; Issazadeh-Navikas, Shohreh

    1997-01-01

    Genetic analysis of experimental autoimmune encephalomyelitis (EAE) can provide clues to the etiology of multiple sclerosis (MS). Identifying the susceptibility genes of DA rats may be particularly rewarding since they are prone to develop a remarkably MS-like chronic and demyelinating disease...

  16. Phospholipase A2 is involved in galactosylsphingosine-induced astrocyte toxicity, neuronal damage and demyelination.

    Directory of Open Access Journals (Sweden)

    Cedric Misslin

    Full Text Available Krabbe disease is a fatal rare inherited lipid storage disorder affecting 1:100,000 births. This illness is caused by mutations in the galc gene encoding for the enzyme galactosylceramidase (GALC. Dysfunction of GALC has been linked to the toxic build-up of the galactolipid, galactosylsphingosine (psychosine, which induces cell death of oligodendrocytes. Previous studies show that phospholipase A2 (PLA2 may play a role in psychosine induce cell death. Here, we demonstrate that non-selective inhibition of cPLA2/sPLA2 and selective inhibition of cPLA2, but not sPLA2, also attenuates psychosine-induced cell death of human astrocytes. This study shows that extracellular calcium is required for psychosine induced cell death, but intracellular calcium release, reactive oxygen species or release of soluble factors are not involved. These findings suggest a cell autonomous effect, at least in human astrocytes. Supporting a role for PLA2 in psychosine-induced cell death of oligodendrocytes and astrocytes, the results show inhibition of PLA2 attenuates psychosine-induced decrease in the expression of astrocyte marker vimentin as well as myelin basic protein (MBP, myelin oligodendrocyte glycoprotein (MOG and the neuronal marker SMI-32 in organotypic slice cultures. These findings provide further mechanistic details of psychosine-induced death of glia and suggest a role for PLA2 in the process. This work also supports the proposal that novel drugs for Krabbe disease may require testing on astrocytes as well as oligodendrocytes for more holistic prediction of pre-clinical and clinical efficacy.

  17. An analysis of autopsy cases of non-Hodgkin lymphoma-with special reference to those masquerading as acute febrile illness.

    Science.gov (United States)

    Saraf, Sonali Rajesh; Naphade, Nilesh S; Kalgutkar, Alka D

    2016-01-01

    As in any medical disorder, in non-Hodgkin lymphomas (NHLs) also, precise analysis of causes of death is needed to focus research efforts and improve morbidity and mortality. The aim of this study was to review the clinical presentation, mode of death and the immunophenotype of the autopsy cases of NHL. Autopsy cases wherein NHL was diagnosed, were selected for study. Autopsy cases which were diagnosed as NHL, either antemortem or after autopsy during a 7 years period at a tertiary care referral centre were studied and reviewed. Descriptive statistical analysis used. The autopsy findings seen in eight cases of NHL were reviewed. Except one, all cases were above 40 years age. Infective etiology (62%) followed by cardiorespiratory failure (38%) was the cause of death observed in these patients. In three cases, the antemortem diagnosis of NHL was missed as the patients were being treated for acute febrile illness and were referred late to the Tertiary Care Centre. One of these was a case of extra-nodal primary splenic lymphoma. As NHL present with nonspecific symptoms, these tumours may not be detected in early stages and hence may not be treated appropriately. These patients have weakened immunity and hence are prone to infection and sepsis which can be a major cause of mortality. This autopsy study experience has shown that NHL can masquerade as acute febrile illness which if not detected early and treated adequately can turn fatal.

  18. DISEASES

    DEFF Research Database (Denmark)

    Pletscher-Frankild, Sune; Pallejà, Albert; Tsafou, Kalliopi

    2015-01-01

    Text mining is a flexible technology that can be applied to numerous different tasks in biology and medicine. We present a system for extracting disease-gene associations from biomedical abstracts. The system consists of a highly efficient dictionary-based tagger for named entity recognition...... of human genes and diseases, which we combine with a scoring scheme that takes into account co-occurrences both within and between sentences. We show that this approach is able to extract half of all manually curated associations with a false positive rate of only 0.16%. Nonetheless, text mining should...... not stand alone, but be combined with other types of evidence. For this reason, we have developed the DISEASES resource, which integrates the results from text mining with manually curated disease-gene associations, cancer mutation data, and genome-wide association studies from existing databases...

  19. Progenitor cell-based treatment of glial disease

    OpenAIRE

    Goldman, Steven A.

    2017-01-01

    Diseases of glia, including both astrocytes and oligodendrocytes, are among the most prevalent and disabling, yet least appreciated, conditions in neurology. In recent years, it has become clear that besides the overtly glial disorders of oligodendrocyte loss and myelin failure, such as the leukodystrophies and inflammatory demyelinations, that a number of neurodegenerative and psychiatric disorders may also be causally linked to glial dysfunction, and derive from astrocytic as well as oligod...

  20. Insulin-like growth factor I treatment reduces demyelination and up-regulates gene expression of myelin-related proteins in experimental autoimmune encephalomyelitis.

    OpenAIRE

    Yao, D L; Liu, X; Hudson, L D; Webster, H D

    1995-01-01

    To compare effects of insulin-like growth factor I (IGF-I) and placebo treatment on lesions that resemble those seen during active demyelination in multiple sclerosis, we induced experimental autoimmune encephalomyelitis in Lewis rats with an emulsion containing guinea pig spinal cord and Freund's adjuvant. On day 12-13, pairs of rats with the same degree of weakness were given either IGF-I or placebo intravenously twice daily for 8 days. After 8 days of placebo or IGF-I (200 micrograms/day o...

  1. Cerebrospinal fluid analysis in infectious diseases of the nervous system: when to ask, what to ask, what to expect

    Directory of Open Access Journals (Sweden)

    Luis dos Ramos Machado

    2013-09-01

    Full Text Available Cerebrospinal fluid (CSF analysis very frequently makes the difference to the diagnosis, not only in relation to infections but also in other diseases of the nervous system such as inflammatory, demyelinating, neoplastic and degenerative diseases. The authors review some practical and important features of CSF analysis in infectious diseases of the nervous system, with regard to acute bacterial meningitis, herpetic meningoencephalitis, neurotuberculosis, neurocryptococcosis, neurocysticercosis and neurosyphilis.

  2. Glial Progenitor Cell–Based Treatment and Modeling of Neurological Disease

    OpenAIRE

    Goldman, Steven A.; Nedergaard, Maiken; Windrem, Martha S.

    2012-01-01

    The diseases of myelin are among the most prevalent and disabling conditions in neurology. These diseases include both the vascular and inflammatory demyelinating disorders of adulthood, as well as the childhood leukodystrophies and cerebral palsy. These fundamentally glial disorders may be amenable to treatment by glial progenitor cells (GPCs), which give rise to astroglia and myelin-producing oligodendrocytes. Given the development of new methods for generating and isolating human GPCs, the...

  3. Atypical idiopathic inflammatory demyelinating lesions (IIDL): Conventional and diffusion-weighted MR imaging (DWI) findings in 42 cases

    Energy Technology Data Exchange (ETDEWEB)

    Koelblinger, Claus; Fruehwald-Pallamar, Julia [Department of Biomedical Imaging and Image-Guided Therapy, Medical University Vienna, Vienna (Austria); Kubin, Klaus [CT/MRI Institut Dr. Klaus Kubin, Salzburg (Austria); Wallner-Blazek, Mirja [Department of Neurology, Medical University Graz, Graz (Austria); Hauwe, Luc van den [Department of Radiology, Medical University of Antwerp, Antwerp (Belgium); Macedo, Leonardo [Department of Radiology, CEDIMAGEM, Centro - Juiz de Fora (Brazil); Puchner, Stefan B. [Department of Biomedical Imaging and Image-Guided Therapy, Medical University Vienna, Vienna (Austria); Thurnher, Majda M., E-mail: majda.thurnher@meduniwien.ac.at [Department of Biomedical Imaging and Image-Guided Therapy, Medical University Vienna, Vienna (Austria)

    2013-11-01

    Introduction: The purpose of this study was to evaluate MR imaging characteristics with conventional and advanced MR imaging techniques in patients with IIDL. Methods: MR images of the brain in 42 patients (20 male, 22 female) with suspected or known multiple sclerosis (MS) from four institutions were retrospectively analyzed. Lesions were classified into five different subtypes: (1) ring-like lesions; (2) Balo-like lesions; (3) diffuse infiltrating lesions; (4) megacystic lesions; and (5) unclassified lesions. The location, size, margins, and signal intensities on T1WI, T2WI, and diffusion-weighted images (DWI), and the ADC values/ratios for all lesions, as well as the contrast enhancement pattern, and the presence of edema, were recorded. Results: There were 30 ring-like, 10 Balo-like, 3 megacystic-like and 16 diffuse infiltrating-like lesions were detected. Three lesions were categorized as unclassified lesions. Of the 30 ring-like lesions, 23 were hypointense centrally with a hyperintense rim. The mean ADC, measured centrally, was 1.50 ± 0.41 × 10{sup −3} mm{sup 2}/s. The mean ADC in the non-enhancing layers of the Balo-like lesions was 2.29 ± 0.17 × 10{sup −3} mm{sup 2}/s, and the mean ADC in enhancing layers was 1.03 ± 0.30 × 10{sup −3} mm{sup 2}/s. Megacystic lesions had a mean ADC of 2.14 ± 0.26 × 10{sup −3} mm{sup 2}/s. Peripheral strong enhancement with high signal on DWI was present in all diffuse infiltrating lesions. Unclassified lesions showed a mean ADC of 1.43 ± 0.13 mm{sup 2}/s. Conclusion: Restriction of diffusion will be seen in the outer layers of active inflammation/demyelination in Balo-like lesions, in the enhancing part of ring-like lesions, and at the periphery of infiltrative-type lesions.

  4. Is Decompressive Surgery for Cervical Spondylotic Myelopathy Effective in Patients Suffering from Concomitant Multiple Sclerosis or Parkinson’s Disease?

    Directory of Open Access Journals (Sweden)

    Taylor E. Purvis

    2017-04-01

    Full Text Available A subset of patients with a demyelinating disease suffer from concurrent cervical spondylotic myelopathy, both of which evince similar symptomatology. Differentiating the cause of these symptoms is challenging, and little research has been done on patients with coexisting diseases. This review explores the current literature on the appropriate surgical management of patients with concurrent multiple sclerosis (MS and cervical spondylotic myelopathy (CSM, and those with both Parkinson’s disease (PD and CSM. MS and CSM patients may benefit from surgery to reduce pain and radiculopathy. Surgical management in PD and CSM patients has shown minimal quality-of-life improvement. Future studies are needed to better characterize demyelinating disease patients with concurrent disease and to determine ideal medical or surgical treatment.

  5. Studies on autoimmune encephalomyelitis in the guinea pig. II. An in vitro investigation on the nature, properties, and specificity of the serum-demyelinating factor.

    Science.gov (United States)

    Lebar, R; Boutry, J M; Vincent, C; Robineaux, R; Voisin, G A

    1976-05-01

    Complement-dependent demyelinating activity of whole brain homogenate (WBH)-induced experimental allergic encephalomyelitis (EAE) sera was tested on long term tissue cultures of in vitro myelinated fetal guinea pig cerebellum. Complement-fixing (CF) auto-antibodies were shown to be the responsible agents, as demonstrated in experiments where all reagents belonged to the same species: guinea pigs of outbred (Hartley) and even of inbred (S2 or S13) strains. These antibodies were of the IgG2 class as shown by Sephadex G-200 and DEAE cellulose fractionation experiments. The corresponding auto-antigen was present in the homogenate and myelin of the central nervous system (CNS) tissue. It was different from the encephalitogenic basic protein of CNS myelin (BP), as shown in experiments where the demyelinating auto-antibodies were induced, detected, and absorbed by WBH or by CNS myelin but not by BP. They were neither induced by nor cross-reacting with cerebroside and peripheral nervous system (PNS) tissue.

  6. Interferon beta-1a in chronic inflammatory demyelinating polyneuropathy: case report Interferon beta en polineuropatía crónica inflamatoria desmienlinizante: caso clínico

    Directory of Open Access Journals (Sweden)

    Andrés Maria Villa

    2004-09-01

    Full Text Available Chronic inflammatory demyelinating polyneuropathy (CIDP is an acquired immune-mediated neuropathy. It presents with a course of progression which may be slow and steady or step-wise or relapsing. Sensory ataxic polyneuropathy may be the only clinical manifestation of this disease. Treatment with interferon beta1a (INF beta1a has been tried with different results in patients who were refractory to other, more conventional, immunomodulatory therapies. Here we report on a patient who had a relapsing form of pure sensory ataxic CIDP and who failed to respond to intravenous human immunoglobulin. He was put on INF beta1a for 3 years. During this period he suffered no relapses while his condition stabilized.La polineuropatía crónica inflamatoria desmielinizante (PCID es una neuropatía inmuno-mediada, que presenta un curso clínico primariamente progresivo o en forma de recaídas. Las manifestaciones sensoriales pueden ser su unica forma de expresión clínica. El tratamiento con interferon beta 1a (IFN beta1a ha sido ensayado en varias oportunidades, con diferentes respuestas terapéuticas, en pacientes refractarios a las terapias inmunomoduladoras convencionales. Nosotros comunicamos un paciente con una forma ataxica recurrente de PCID, que no respondió al tratamiento con inmunoglobulina endovenosa. Posteriormente fue tratado con IFN beta 1 a por tres años. Durante el período de seguimiento no mostró nuevas recaídas y su cuadro neurológico se estabilizó.

  7. The value of qualitative and quantitative assessment of lesion to cerebral cortex signal ratio on double inversion recovery sequence in the differentiation of demyelinating plaques from non-specific T2 hyperintensities

    Energy Technology Data Exchange (ETDEWEB)

    Hamcan, Salih; Battal, Bilal; Akgun, Veysel; Sari, Sebahattin; Tasar, Mustafa [Gulhane Military Medical School, Department of Radiology, Etlik, Ankara (Turkey); Oz, Oguzhan; Tasdemir, Serdar [Gulhane Military Medical School, Department of Neurology, Ankara (Turkey); Bozkurt, Yalcin [Golcuk Military Hospital, Department of Radiology, Kocaeli (Turkey)

    2017-02-15

    To assess the usefulness of the visual assessment and to determine diagnostic value of the lesion-to-cerebral cortex signal ratio (LCSR) measurement in the differentiation of demyelinating plaques and non-specific T2 hyperintensities on double inversion recovery (DIR) sequence. DIR and fluid-attenuated inversion recovery (FLAIR) sequences of 25 clinically diagnosed multiple sclerosis (MS) patients and 25 non-MS patients with non-specific T2-hyperintense lesions were evaluated visually and LCSRs were measured by two observers independently. On DIR sequence, the calculated mean LCSR ± SD for demyelinating plaques and non-specific T2-hyperintense lesions were 1.60 ± 0.26 and 0.75 ± 0.19 for observer1, and 1.61 ± 0.27 and 0.74 ± 0.19 for observer2. LCSRs of demyelinating plaques were significantly higher than other non-specific T2-hyperintense lesions on DIR sequence. By using the visual assessment demyelinating plaques were differentiated from non-specific T2-hyperintensities with 92.8 % sensitivity, 97.5 % specificity and 95.1 % accuracy for observer1 and 92.8 % sensitivity, 95 % specificity and 93.9 % accuracy for observer2. Visual assessment and LCSR measurement on DIR sequence seems to be useful for differentiating demyelinating MS plaques from supratentorial non-specific T2 hyperintensities. This feature can be used for diagnosis of MS particularly in patients with only supratentorial T2-hyperintense lesions who are categorized as radiologically possible MS. (orig.)

  8. A randomised, double-blinded, placebo controlled trial of the effect of subcutaneous immunoglobulin on muscular performance in chronic inflammatory demyelinating polyneuropathy

    DEFF Research Database (Denmark)

    Jakobsen, Johannes Klitgaard; Markvardsen, Lars Høj; Harbo, Thomas

    Objective: We hypothesised that the effect of subcutaneous infusion of immunoglobulins(SCIG) on muscular performance in chronic inflammatory demyelinating polyneuropathy(CIDP) is superior to that of placebo and equals the therapeutic effect of intravenous infusion(IVIG). Background Subcutaneous...... treatment with large amounts of immunoglobulins in multifocal motor neuropathy is feasible, safe and effective. In CIDP case reports indicate its therapeutic usefullness as well. Design/Methods: Subjects in IVIG maintenace therapy full-filling the EFNS/PNS criteria for CIDP were considered for participation......G at a concentration of 1.6g/10cc or subcutaneous saline in a double-blinded fashion. Infusions were given twice or thrice weekly for 12 weeks at home. The amount of immunoglobulin corresponded to the clinically predetermined dose. The first subcutaneous infusion was delivered two weeks after the last IVIG treatment...

  9. Parkinson's disease associated with impaired oxidative phosphorylation

    International Nuclear Information System (INIS)

    Finsterer, J.; Jarius, C.; Baumgartner, M.

    2001-01-01

    Parkinson's disease may be due to primary or secondary oxidative phosphorylation (OXPHOS) defects. In a 76-year-old man with Parkinson's disease since 1992, slightly but recurrently elevated creatine phosphokinase, recurrently elevated blood glucose, thickening of the left ventricular myocardium, bifascicular block and hypacusis were found. Cerebral MRI showed atrophy, periventricular demyelination, multiple, disseminated, supra- and infratentorial lacunas, and haemosiderin deposits in both posterior horns. Muscle biopsy showed typical features of an OXPHOS defect. Whether the association of Parkinson's disease and impaired OXPHOS was causative or coincidental remains unknown. Possibly, the mitochondrial defect acted as an additional risk factor for Parkinson's disease or the OXPHOS defect worsened the preexisting neurological impairments by a cumulative or synergistic mechanism. In conclusion, this case shows that Parkinson's disease may be associated with a mitochondrially or nuclearly encoded OXPHOS defect, manifesting as hypacusis, myopathy, axonal polyneuropathy, cardiomyopathy and recurrent subclinical ischaemic strokes and haemorrhages. (orig.)

  10. Canine distemper virus persistence in demyelinating encephalitis by swift intracellular cell-to-cell spread in astrocytes is controlled by the viral attachment protein.

    Science.gov (United States)

    Wyss-Fluehmann, Gaby; Zurbriggen, Andreas; Vandevelde, Marc; Plattet, Philippe

    2010-05-01

    The mechanism of viral persistence, the driving force behind the chronic progression of inflammatory demyelination in canine distemper virus (CDV) infection, is associated with non-cytolytic viral cell-to-cell spread. Here, we studied the molecular mechanisms of viral spread of a recombinant fluorescent protein-expressing virulent CDV in primary canine astrocyte cultures. Time-lapse video microscopy documented that CDV spread was very efficient using cell processes contacting remote target cells. Strikingly, CDV transmission to remote cells could occur in less than 6 h, suggesting that a complete viral cycle with production of extracellular free particles was not essential in enabling CDV to spread in glial cells. Titration experiments and electron microscopy confirmed a very low CDV particle production despite higher titers of membrane-associated viruses. Interestingly, confocal laser microscopy and lentivirus transduction indicated expression and functionality of the viral fusion machinery, consisting of the viral fusion (F) and attachment (H) glycoproteins, at the cell surface. Importantly, using a single-cycle infectious recombinant H-knockout, H-complemented virus, we demonstrated that H, and thus potentially the viral fusion complex, was necessary to enable CDV spread. Furthermore, since we could not detect CD150/SLAM expression in brain cells, the presence of a yet non-identified glial receptor for CDV was suggested. Altogether, our findings indicate that persistence in CDV infection results from intracellular cell-to-cell transmission requiring the CDV-H protein. Viral transfer, happening selectively at the tip of astrocytic processes, may help the virus to cover long distances in the astroglial network, "outrunning" the host's immune response in demyelinating plaques, thus continuously eliciting new lesions.

  11. Magnetic Resonance Finding of Acute Marchiafava-Bignami Disease with Diffuse Involvement: A Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Heo, Young Jin; Jeong, Hae Woong; In, Hyun Sin [Dept. of Radiology, Busan Paik Hospital, Inje University College of Medicine, Busan (Korea, Republic of)

    2011-11-15

    Marchiafava-Bignami disease (MBD) is a rare toxic disorder strongly associated with chronic alcoholism. It is characterized by progressive demyelination and necrosis of the corpus callosum. The process may extend to neighboring white matter and subcortical regions. We report a case of MBD in which fluid-attenuated inversion recovery and diffusion-weighted imaging revealed symmetrical hyperintense lesions with diffuse involvement of the corpus callosum, white matter, corticospinal tract, internal capsule, and middle cerebellar peduncle.

  12. Low grade papillary transitional cell carcinoma pelvic recurrence masquerading as high grade invasive carcinoma, ten years after radical cystectomy

    Directory of Open Access Journals (Sweden)

    Mortazavi Amir

    2008-09-01

    Full Text Available Abstract Background Tumor recurrence following radical cystectomy for a low-grade superficial transitional cell carcinoma (TCC is exceedingly uncommon and has not been reported previously. Case presentation We describe a case of a young male presenting with anorexia, weight loss and a large, painful locally destructive pelvic recurrence, ten years after radical cystoprostatectomy. The pathology was consistent with a low-grade urothelial carcinoma. After an unsuccessful treatment with cisplatin-based chemotherapy, the patient underwent a curative intent hemipelvectomy with complete excision of tumor and is disease free at one year follow-up. Conclusion A literature review related to this unusual presentation is reported and a surgical solutions over chemotherapy and radiotherapy is proposed.

  13. Peripheral tuberculous lymphadenitis masquerading as metastatic gastric carcinoma on F 18 FDG dual time point PET/CT

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Su Kon; Shin, Jeong Eun; Lee, Jai Hyuen [Dankook Univ. College of Medicine, Cheonan (Korea, Republic of)

    2012-12-15

    A 59 year old woman was hospitalized with weight loss, anorexia, and low grade fever for 2 weeks. she had under gone a total gastrectomy followed by adjuvant chemotherapy 10 years prior due to advanced gastric cancer to evaluate recurrence, fluorine 19 fluorodeoxyglucose (FDG) dual time point positron emission tomography/computed tomography (DTPP) was conducted with a gemini GXL 6 PET/CT system (Philips, Hamburg, Germany). FDG DTPP was performed twice, with an early scan 60 min after FDG injection and a delayed scan 150min after FDG injection. The early scan showed that multiple hypermetabolic lesions were located along left supraclavicular, mediastinal (1R, 2R, 3, 4R and 7) and left paraaortic (retroperitoneal) nodal stations. The delayed scan showed that all the above mentioned lesions revealed a more increased maximal standard uptake value (SUVmax) than was detected in the early scan. both scans did not show intrapulmonary lesion. For example, the left supraclavicular lymph node, 2R and retroperitoneal lymph node were observed with early and delayed SUV maxes of 4.5/7.6/3.6 and 6.7/7.7/3.6 and 6.7/10.7/4.4, respectively. Tom confirm diagnosis and establish a treatment plan, a mediastinoscopic biopsy of one of the mediastinal lymph nodes (4R) was subsequently performed and tuberculosis was ultimately diagnosed. Then, the patient started an antituberculosis treatment instead of antichemotherapy. A follow up FDG PET/CT performed 12 months later showed disappeared FDG uptake and significant decreased change of multiple peripheral tuberculous lymphadenitis. Whole body FDG PET/CT has widespread use in diagnosing and staging variable malignancies, and plays an important role in detection of gastric cancer recurrence. However, FDG is not cancer specific and some inflammatory diseases have shown higher FDG uptake on PET imaging. Infection, inflammation, and granulomatosis are also known to cause false positive FDG PET scans because activated inflammatory cells show

  14. Intimal spindle cell sarcoma masquerading as adult-onset symptomatic pulmonic stenosis: a case report and review of the literature.

    Science.gov (United States)

    Manmadhan, Arun; Malhotra, Sunil P; Weinberg, Catherine R; Reyentovich, Alex; Latson, Larry A; Bhatla, Puneet; Saric, Muhamed

    2017-10-30

    Pulmonary artery intimal spindle cell sarcomas are rare and carry with them a poor prognosis and high rate of recurrence. In extremely rare cases, this tumor can infiltrate the pulmonic valve and manifest as adult-onset pulmonic stenosis. We report an unusual case of a patient with symptomatic, adult-onset severe pulmonic stenosis who was referred for possible balloon valvuloplasty but was subsequently found to have pulmonary artery intimal sarcoma infiltrating the pulmonary valve leading to progressive exertional dyspnea. The presence of adult-onset pulmonic stenosis should prompt the clinician to investigate further as most cases of pulmonic stenosis are congenital in nature and present early in life. Careful diagnostic evaluation in concert with multimodal imaging should take place to arrive at the correct and challenging diagnosis of sarcoma-induced adult-onset severe pulmonic stenosis. Given the poor prognosis and rapid progression of disease, early diagnosis is crucial.

  15. Serous Tubal Intraepithelial Carcinoma or Not? Metastases to Fallopian Tube Mucosa Can Masquerade as In Situ Lesions.

    Science.gov (United States)

    Singh, Reena; Cho, Kathleen R

    2017-10-01

    - Nonuterine high-grade serous carcinomas (HGSCs) are believed to arise most often from precursors in the fallopian tube referred to as serous tubal intraepithelial carcinomas (STICs). A designation of tubal origin has been suggested for all cases of nonuterine HGSC if a STIC is identified. - To highlight that many different types of nongynecologic and gynecologic carcinomas, including HGSC, can metastasize to the tubal mucosa and mimic de novo STIC. - A mini-review of several recently published studies that collectively examine STIC-like lesions of the fallopian tube. - The fallopian tube mucosa can be a site of metastasis from carcinomas arising elsewhere, and pathologists should exercise caution in diagnosing STIC without first considering the possibility of metastasis. Routinely used immunohistochemical stains can often be used to determine if a STIC-like lesion is tubal or nongynecologic in origin. In the context of uterine and nonuterine HGSC, STIC may represent a metastasis rather than the site of origin, particularly when widespread disease is present.

  16. Chronic GM2 gangliosidosis masquerading as atypical Friedreich ataxia: clinical, morphologic, and biochemical studies of nine cases.

    Science.gov (United States)

    Willner, J P; Grabowski, G A; Gordon, R E; Bender, A N; Desnick, R J

    1981-07-01

    A progressive spinocerebellar degenerative disorder was characterized in nine patients, aged 11 to 37 years, from four unrelated Ashkenazi Jewish families; affected individuals had markedly deficient beta-hexosaminidase A activity. Symptoms included early onset of cerebellar signs (tremor, incoordination, and dysarthia) and, with maturity, the development of upper and lower motor neuron disorders, marked dysarthia, and ataxia. Three older patients, aged 26, 32, and 37 years, had dementia or recurrent psychotic episodes. Membrane-bound lamellar cytoplasmic inclusions, consistent with lysosomal ganglioside accumulation, were observed in rectal ganglia. The activity of beta-hexosaminidase A was markedly deficient in all sources analyzed. Parents had activities consistent with heterozygosity, confirming autosomal-recessive transmission of the beta-hexosaminidase A-deficient gene and the adult variant disorder. Residual beta-hexosaminidase A activity, partially purified by anion-exchange chromatography from cultured skin fibroblasts of the affected individuals, was heat-labile and co-electrophoresed with normal beta-hexosaminidase A. These findings suggest that these patients were allelic for a new beta-hexosaminidase A mutation and may represent a genetic compound of this allele and the allele causing Tay-Sachs disease.

  17. ROLE OF MRI IN WHITE MATTER DISEASES- CLINICO-RADIOLOGICAL CORRELATION

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    Ravindranath Reddy Kamireddy

    2017-11-01

    Full Text Available BACKGROUND The diagnostic process is difficult as there are many different white matter disorders (inherited and acquired. MRI has high diagnostic specificity to study the pattern of brain structures. MRI is more useful in demonstrating abnormalities of myelination. MATERIALS AND METHODS Our study developed a practical algorithm that relies mainly on the characteristics of brain MRI. Our study included clinicallysuspected patients with demyelination during a period of one year. RESULTS Our study included 25 clinically-suspected patients (out of total of 400 patients with demyelination during a period of one year (February 2016 to January 2017.  Multiple sclerosis accounted for the majority of cases (36.0% followed by acute disseminated encephalomyelitis (20%.  In multiple sclerosis, majority of the patients presented in the third decade of life with a definite female preponderance (M:F-1:2.  The most common symptom and site of involvement were visual impairment (73.3% and periventricular area (80%, respectively.  Other causes like PML, PVL, CPM, reversible posterior leucoencephalopathy, leukodystrophies and motor neuron disease comprised the remainder of the cases. CONCLUSION MRI due to its excellent grey white matter resolution is very sensitive in detecting subtle demyelination, the sensitivity being still further enhanced by FLAIR sequences. MRI in correlation with the clinical signs and symptoms is an ideal modality in early diagnosis of white matter diseases.

  18. Effects of chlorogenic acid on adenine nucleotides hydrolyzing enzyme activities and expression in platelets of rats experimentally demyelinated with ethidium bromide.

    Science.gov (United States)

    Leal, Claudio A M; Leal, Daniela B R; Adefegha, Stephen A; Morsch, Vera M; Beckmann, Diego V; Castilhos, Lívia G; Thorstenberg, Maria L P; Jaques, Jeandre A Dos S; Souza, Viviane do C G; Farias, Júlia G; Martins, Caroline C; Schetinger, Maria R C

    2016-07-01

    The effects of chlorogenic acid (one of the major phenolic acid found in human diets) were investigated on the adenine nucleotides hydrolyzing enzymes; ecto-nucleotide pyrophosphatase/phophodiesterase (E-NPP), ecto-nucleoside triphosphate diphosphohydrolase (E-NTPDase), E-5'- nucleotidase and ecto-adenosine deaminase (E-ADA) activities and expression in platelets of rats experimentally demyelinated with ethidium bromide. Rats were divided into four groups of eight animals each. Group I rats were control rats; injected with saline (CT), group II rats were injected with saline and treated with chlorogenic acid (AC), group III rats were injected with 0.1% ethidium bromide (EB) and group IV rats were injected with 0.1% EB and treated with chlorogenic acid (EB+AC). The activities of the enzymes were analyzed using colorimetric methods, and the gene expression of NTPDase 1, 2 and 3 were analyzed using the polymerase chain reaction (PCR). The results revealed that there was a significant (Pchlorogenic acid significantly (P0.05) change was observed in the E-NPP activity of EB+AC group (2.19±0.08nmol p-nitrophenol released/min/mg protein) when compared to CT group (2.33±0.14nmol p-nitrophenol released/min/mg protein). In addition, there was a significant (P0.05) difference was observed in AMP hydrolysis between AC, AC+EB and CT groups. Conversely, there were no significant (P>0.05) differences in ATP and ADP hydrolyses between all the groups (AC, EB, AC+EB and CT groups). Likewise, there were no significant (P>0.05) changes in E-ADA activity and percentage platelet aggregation among all groups studied. Similarly, no significant (P>0.05) change was observed in the expression of E-NTPDase 1, 2 and 3 in all the groups tested. Our study revealed that chlorogenic acid may modulate the hydrolysis of adenine nucleotides in platelets of rats demyelinated and treated with chlorogenic acid via alteration of E-NPP and ecto-5'-nucleotidase activities. Copyright © 2016 Elsevier

  19. Misdiagnosis of early Lyme disease as the summer flu

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    John N. Aucott

    2011-09-01

    Full Text Available Lyme disease is often identified by the hallmark erythema migrans rash, but not all early cases present with a rash. In other cases the rash may be unseen or unrecognized by a physician. In these situations, Lyme disease is difficult to diagnose because it masquerades as a non-specific viral-like illness. The seasonal peak of Lyme disease ranging from May through September overlaps with that of viral illnesses such as enteroviral infections, West Nile virus, and in rare years such as 2009, early influenza season. We present a case of a patient with Lyme disease who was initially misdiagnosed with influenza A during the summer of 2009. Because of the diagnostic importance of recognizing the erythema migrans rash, physicians in endemic regions should always ask about new rashes or skin lesions and perform a thorough physical examination when patients present over the summer with viral-like symptoms. Even when no rash is evident, Lyme disease should be considered if these symptoms persist or worsen without a specific diagnosis.

  20. 1.5-T high-resolution MR imaging of oculomotor disturbances due to intrinsic brain-stem disease

    International Nuclear Information System (INIS)

    Patel, S.C.; Quint, D.J.; Sanders, W.P.; Mehta, B.A.; Boulos, R.S.; Froelich, J.W.

    1987-01-01

    Seventeen patients who presented with oculomotor disturbances (internuclear ophthalmoplegia, ophthalmoparesis) underwent MR imaging, which demonstrated brain-stem abnormalities in 11 cases. Lesions identified included occult vascular malformation with hemorrhage (two), hypertensive hemorrhage (one), infarction (two), neoplasm (two), and demyelinating disease (four). The authors' exhibit illustrates the exquisite anatomic detail displayed by high-field MR imaging in localizing various intrinsic brain-stem lesions in the region of the third, fourth, and sixth cranial nuclei and the medial longitudinal fasciculus

  1. Computed tomography and MRI in Krabbe's disease

    International Nuclear Information System (INIS)

    Winants, D.; Bernard, C.; Galloy, M.A.; Hoeffel, J.C.; Vidailhet, M.

    1988-01-01

    Krabbe's disease whose CT appearance is well known should benefit of the development of MRI as this method is more accurate. MRI permits early diagnosis of leucodystrophy. Yet it must be emphasized that abnormal white matter patterns are not sufficient to permit the diagnosis of Krabbe disease. In the case reported atrophy of the brain, the cerebellum and the brain-stem, demyelination of the white matter and necrosis of corpus callosum were observed. The abnormalities of the brain on MRI pictures help in the diagnosis as they may alert clinicians to the possibility of Krabbe disease in infants with progressive encephalopathy. A definitive diagnosis can be established thanks to the laboratory tests [fr

  2. CHARCOT-MARIE-TOOTH DISEASE

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    Lea Leonardis

    2003-09-01

    Full Text Available Background. Charcot-Marie-Tooth (CMT disease is a common inherited disorder of the peripheral nervous system. In our paper, different types of CMT are described with their typical clinical pictures, electrophysiological signs and molecular genetic studies. CMT is classified as demyelinative and axonal type and distal motor neuronopathy.Conclusions. CMT can be of autosomal dominant, recessive and X-linked inheritance. The most frequent form of CMT is the result of the dominantly inherited duplication of chromosome 17p11.2 and is marked as CMT1A. The same group involves also rare patients with point mutation in the peripheral myelin protein-22 gene. CMT1B is associated with point mutations in protein zero gene. CMT1C is linked to chromosome 16p13.1–12.3. Patients with point mutations in early growth response 2 gene (EGR2 are included in group CMT1D. The disease can be also inhereted X-linked (CMTX with the mutations in connexin-32 gene. In autosomal recessive inherited demyelinating polyneuropathies (CMT4, mutations are found in the myotubularin-related protein-2 (CMT4B, N-myc downstream-regulated gene 1 (CMT4D, EGR2 (CMT4E, and in the periaksin (CMT4F genes. In axonal inherited neuropathy, mutations are found in KIF1beta (CMT2A and in light neurofilament (CMT2E genes, other forms map to different chromosomal loci (CMT2B, CMT2D, CMT2F. Some suggestions for the diagnostic procedures of patients with CMT are given.

  3. Early electrophysiological findings in acute inflammatory demyelinating polyradiculoneuropathy variant of Guillain-Barre syndrome in the Pakistani population - a comparison with global data.

    Science.gov (United States)

    Wali, Ahmad; Kanwar, Dureshahwar; Khan, Safoora A; Khan, Sara

    2017-12-01

    Acute inflammatory demyelinating polyradiculoneuropathy (AIDP) and acute motor axonal neuropathy are the most common variants of Guillian-Barre syndrome documented in the Asian population. However, the variability of early neurophysiologic findings in the Asian population compared to western data has not been documented. Eighty-seven cases of AIDP were retrospectively reviewed for their demographic, clinical, electrophysiological, and laboratory data. Mean age of subjects was 31 ± 8 years with males more commonly affected. Motor symptoms (97%) at presentation predominated. Common early nerve conduction findings included low motor amplitudes (85%), recordable sural sensory responses (85%), and absent H-reflex responses (65%). Prolonged F-latencies were found most commonly in posterior tibial nerves (23%) in the lower limbs and median and ulnar nerves (18%) in the upper limbs. Blink reflex (BR) studies were performed in 57 patients and were abnormal in 80% of those with clinical facial weakness and in 17 of 52 patients (33%) with no clinical cranial nerve signs, suggesting subclinical cranial nerve involvement. Abnormal motor and sensory amplitudes are seen early. Prolonged distal latencies, temporal dispersion/conduction blocks and sural sparing pattern are other common early nerve conduction study findings of AIDP seen in the Pakistani population. There are no significant differences in abnormalities of conduction velocities and delayed reflex responses compared to published data. The BR can help in the early diagnosis of AIDP. © 2017 Peripheral Nerve Society.

  4. Antibody-Mediated Rejection of the Heart in the Setting of Autoimmune Demyelinating Polyneuropathy: A Case Report and Review of the Literature

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    Kathryn J. Lindley

    2012-01-01

    Full Text Available Background. Antibody-mediated rejection (AMR is caused by the production of donor-specific antibodies (DSA which lead to allograft injury in part via complement activation. The inflammatory demyelinating polyneuropathies (IDP are inflammatory disorders of the nervous system, involving both cellular and humoral immune mechanisms directed against myelin. Case Report. A 58-year-old man five years after heart transplant presented with progressive dyspnea, imbalance, dysphagia, and weakness. Nerve conduction studies and electromyogram were consistent with IDP. Plasmapheresis and high-dose steroids resulted in improvement in neurologic symptoms. Within two weeks, he was readmitted with anasarca and acute renal failure, requiring intravenous furosemide and inotropic support. Echocardiogram and right heart catheterization revealed reduced cardiac function and elevated filling pressures. DSA was positive against HLA DR53, and endomyocardial biopsy revealed grade 1R chronic inflammation, with strong capillary endothelial immunostaining for C4d. Plasmapheresis and intravenous immunoglobulin (IVIG were initiated. His anasarca and renal failure subsequently resolved, echocardiogram showed improved function off inotropes, and anti-DR53 MFI was reduced by 57%. Conclusions. This is an example of a single immune-mediated process causing concurrent IDP and AMR. The improvement in cardiac function and neurologic symptoms with plasmapheresis, IVIG, and high-dose steroids argues for a unifying antibody-mediated mechanism.

  5. Inflammatory bowel disease: an increased risk factor for neurologic complications.

    Science.gov (United States)

    Morís, Germán

    2014-02-07

    Only a very few systematic studies have investigated the frequency of neurologic disorders in patients with Crohn's disease (CD) and ulcerative colitis (UC), which are the two main types of inflammatory bowel disease (IBD). Results have been inconsistent and variable, owing to differences in case-finding methods and evaluated outcomes in different studies. The most frequent neurologic manifestations reported in CD and UC populations are cerebrovascular disease (with either arterial or venous events), demyelinating central nervous system disease, and peripheral neuropathy (whether axonal or demyelinating); however, the literature describes numerous nervous system disorders as being associated with IBD. The pathogenesis of nervous system tissue involvement in IBD has yet to be elucidated, although it seems to be related to immune mechanisms or prothrombotic states. The recently-introduced tumor necrosis factor (TNF) inhibitors have proven successful in controlling moderate to severe IBD activity. However, severe neurologic disorders associated with TNF inhibitors have been reported, which therefore raises concerns regarding the effect of anti-TNF-α antibodies on the nervous system. Although neurological involvement associated with IBD is rarely reported, gastroenterologists should be aware of the neurologic manifestations of IBD in order to provide early treatment, which is crucial for preventing major neurologic morbidity.

  6. Sacral lipomyelomeningocele masquerading as sacrococcygeal ...

    African Journals Online (AJOL)

    Spina bifida is a common neurosurgical congenital anomaly. The spectrum is wide including rare anomaly such as lipomyelomeningocele. Sacral lipomyelomeningocele may simulate sacrococcygeal teratoma thereby causing a diagnostic dilemma. This may pose challenges during management. This a report of a child ...

  7. Coccidioidomycosis Masquerading as Eosinophilic Ascites

    Directory of Open Access Journals (Sweden)

    Kourosh Alavi

    2015-01-01

    Full Text Available Endemic to the southwestern parts of the United States, coccidioidomycosis, also known as “Valley Fever,” is a common fungal infection that primarily affects the lungs in both acute and chronic forms. Disseminated coccidioidomycosis is the most severe but very uncommon and usually occurs in immunocompromised individuals. It can affect the central nervous system, bones, joints, skin, and, very rarely, the abdomen. This is the first case report of a patient with coccidioidal dissemination to the peritoneum presenting as eosinophilic ascites (EA. A 27-year-old male presented with acute abdominal pain and distention from ascites. He had eosinophilia of 11.1% with negative testing for stool studies, HIV, and tuberculosis infection. Ascitic fluid exam was remarkable for low serum-ascites albumin gradient (SAAG, PMN count >250/mm3, and eosinophils of 62%. Abdominal imaging showed thickened small bowel and endoscopic testing negative for gastric and small bowel biopsies. He was treated empirically for spontaneous bacterial peritonitis, but no definitive diagnosis could be made until coccidioidal serology returned positive. We noted complete resolution of symptoms with oral fluconazole during outpatient follow-up. Disseminated coccidioidomycosis can present in an atypical fashion and may manifest as peritonitis with low SAAG EA. The finding of EA in an endemic area should raise the suspicion of coccidioidal dissemination.

  8. Extramedullary pasmacytoma masquerading as chalazion.

    Science.gov (United States)

    Maheshwari, Rajat; Maheshwari, Sejal

    2009-01-01

    Extramedullary plasmacytoma (EMP) is a rare entity that belongs to the category of non-Hodgkin lymphoma. EMP make up 4% of all plasma cell tumors and occur mainly in the upper aerodigestive tract. Primary plasmacytoma involving the orbit is rare. There is a relation between solitary plasmacytoma and subsequent development of multiple myeloma. As no predictors of progression have been identified patients need indefinite follow-up. The authors report a case of EMP involving the eyelid in an otherwise healthy adult female.

  9. Apotemnophilia masquerading as medical morbidity.

    Science.gov (United States)

    Bensler, J Mike; Paauw, Douglas S

    2003-07-01

    We report a case of apotemnophilia, or "love of amputation," in a man in his mid-20s. Apotemnophilia is defined as self-desired amputation driven by the patient's erotic fantasy of possessing an amputated limb and overachieving despite being handicapped. The desire of a patient with apotemnophilia for amputation is obsessive, and a history of repeated, unexplained injuries to the same segment of the body is common among these patients. Patients with apotemnophilia secretly harm themselves to necessitate amputation of an injured limb, which creates a diagnostic challenge for the health care provider because of the atypical presentation of self-inflicted medical morbidity caused by apotemnophilia.

  10. Wegener granulomatosis masquerading as pneumonia.

    Science.gov (United States)

    Theodorou, D J; Theodorou, S J; Mpougias, K; Mastora, M; Stefanaki, S; Akritidis, N C

    2010-01-01

    We report a case of an elderly patient with a limited form of Wegener granulomatosis, which simulated the clinical and imaging features of organizing pneumonia. Here we call attention to this atypical case presentation that eloquently illustrates the many faces of Wegener granulomatosis.

  11. Global Drums and Local Masquerades

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    Liwhu Betiang

    2013-12-01

    Full Text Available TV broadcasting has been in Nigeria for more than 50 years (1959-2009. Its development has brought about a series of local responses to global socioeconomic and political environments and “soft” stimuli. This conclusion is based on a critical, interpretive reading of the history, form, and content of television in Nigeria from Obafemi Awolowo’s Western Nigeria Television in Ibadan through the federal government’s reactive establishment of the national network: the Nigeria Television Authority, and later, states and private television stations. The ultimate deregulation of television broadcasting in 1992, perceived as Babangida’s “politically-correct” reaction to the pressures from the Bretton Woods institutions, opened up national media markets for global penetration, and fast-tracked media globalization and its effects in Nigeria. While television stations in Nigeria have multiplied in numerical terms, programming/content/form have followed the global market/technological determinism turning Nigerian TV into localized versions of commercialized western master-scripts with very little local ideological direction.

  12. Porokeratosis Masquerading As Lupus Vulgaris

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    Das Sudip

    2004-01-01

    Full Text Available Porokeratosis is a specific disorder of kerstinization characterized histologically by cornoid lamella. Lesions of porokeratosis present varied features, often mimicking other dermatological disorders. Herein, we report a case of porokeratosis of Mibelli presenting with verrucous lesions simulating lupus vulgaris.

  13. Lipoproteinlipase activity in multiple sclerosis and other neurologic diseases.

    Science.gov (United States)

    Popoviciu, L; Hobai, S; Condrat, A

    1990-01-01

    Taking into account the disturbances of lipid metabolism occurring in patients with demyelinating diseases, an expected finding was their increased basal lipolytic activity. We investigated 135 cases divided into 4 categories: 1. MS; 2. inflammatory diseases of the CNS; 3. cerebrovascular diseases and other diseases without any CNS organic involvements; 4. a group of absolutely healthy controls (blood donors). High values of plasmatic lipolytic activity were found in multiple sclerosis patients as compared with the other groups. They were probably due to the effect of a disturbance with an influence upon the basal lipoproteinlipase activity too. This influence is likely to refer to the stages of the process by which heparin releases lipoprotein-lipase from the capillary endothelium. Significant correlations between the clinical evolution of the disease and the values of lipolytic activity were pointed out. Measurement of this activity is a useful biochemical test in the diagnosis of multiple sclerosis.

  14. Subcutaneous immunoglobulin as first-line therapy in treatment-naive patients with chronic inflammatory demyelinating polyneuropathy: randomized controlled trial study.

    Science.gov (United States)

    Markvardsen, L H; Sindrup, S H; Christiansen, I; Olsen, N K; Jakobsen, J; Andersen, H

    2017-02-01

    Subcutaneous immunoglobulin (SCIG) is effective as maintenance treatment in chronic inflammatory demyelinating polyneuropathy (CIDP). We investigated whether multiple subcutaneous infusions are as effective as conventional therapy with intravenous loading doses in treatment-naive patients with CIDP. Twenty patients fulfilling the clinical and electrophysiological criteria for CIDP were included and treated with either SCIG (0.4 g/kg/week) for 5 weeks or intravenous immunoglobulin (IVIG) (0.4 g/kg/day) for 5 days. After 10 weeks, patients were switched to the opposite treatment arm and followed for a further 10 weeks. All participants were evaluated at weeks 0, 2, 5 and 10 during both therapies. Primary outcome was combined isokinetic muscle strength (cIKS). Secondary outcomes were disability, clinical evaluation of muscle strength and the performance of various function tests. All participants received both therapies, 14 completing the protocol. Overall, cIKS increased by 7.4 ± 14.5% (P = 0.0003) during SCIG and by 6.9 ± 16.8% (P = 0.002) during IVIG, the effect being similar (P = 0.80). Improvement of cIKS peaked 2 weeks after IVIG and 5 weeks after SCIG. Disability improved during SCIG treatment only. Muscle strength determined by manual muscle testing improved after 5 and 10 weeks during SCIG but only after 5 weeks during IVIG. The remaining parameters improved equally during both treatments. Plasma immunoglobulin G levels at baseline and improvement of cIKS were related. In treatment-naive patients with CIDP, short-lasting SCIG and IVIG therapy improve motor performance to a similar degree, but with earlier maximal improvement following IVIG than SCIG treatment. © 2016 EAN.

  15. Diffusion tensor imaging can be used to detect lesions in peripheral nerves in patients with chronic inflammatory demyelinating polyneuropathy treated with subcutaneous immunoglobulin

    Energy Technology Data Exchange (ETDEWEB)

    Markvardsen, Lars H.; Andersen, Henning [Aarhus University Hospital, Department of Neurology, Aarhus C (Denmark); Vaeggemose, Michael [Aarhus University Hospital, Department of Neurology, Aarhus C (Denmark); Aarhus University Hospital, Department of Diagnostic Imaging: MR Research Centre, Aarhus (Denmark); Ringgaard, Steffen [Aarhus University Hospital, Department of Diagnostic Imaging: MR Research Centre, Aarhus (Denmark)

    2016-08-15

    Magnetic resonance imaging (MRI) with diffusion tensor imaging (DTI) has shown that fractional anisotropy (FA) is lower in peripheral nerves in chronic inflammatory demyelinating polyneuropathy (CIDP). We examined whether DTI correlates to muscle strength or impairment. MRI of sciatic and tibial nerves was performed on 3-T MR scanner by obtaining T2- and DTI-weighted sequences with fat saturation. On each slice of T2-weighted (T2w) and DTI, the tibial and sciatic nerves were segmented and served for calculation of signal intensity. On DTI images, pixel-by-pixel calculation of FA and apparent diffusion coefficient (ADC) was done. Muscle strength at knee and ankle was determined by isokinetic dynamometry and severity of CIDP by neuropathy impairment score (NIS). Fourteen CIDP patients treated with subcutaneous immunoglobulin were compared to gender- and age-matched controls. T2w values expressed as a nerve/muscle ratio (nT2w) were unchanged in CIDP versus controls 0.93 ± 0.21 versus 1.02 ± 0.21 (P = 0.10). FA values were lower in CIDP compared to controls 0.38 ± 0.07 versus 0.45 ± 0.05 (P < 0.0001), and ADC values were higher in CIDP versus controls 1735 ± 232 versus 1593 ± 116 x 10{sup -6} mm{sup 2}/s (P = 0.005). In CIDP, FA values correlated to clinical impairment (NIS) (r = -0.57, P = 0.03), but not to muscle strength. FA value in the sciatic nerve distinguishes CIDP from controls with a sensitivity and a specificity of 92.9 %. CIDP patients have unchanged nT2w values, lower FA values, and higher ADC values of sciatic and tibial nerves compared to controls. FA values correlated to NIS but were unrelated to muscle strength. DTI of sciatic nerves seems promising to differentiate CIDP from controls. (orig.)

  16. ELR chemokine signaling in host defense and disease in a viral model of central nervous system disease

    Directory of Open Access Journals (Sweden)

    Martin P Hosking

    2014-06-01

    Full Text Available Intracranial infection of the neurotropic JHM strain of mouse hepatitis virus (JHMV into the central nervous system (CNS of susceptible strains of mice results in an acute encephalomyelitis, accompanied by viral replication in glial cells and robust infiltration of virus-specific T cells that contribute to host defense through cytokine secretion and cytolytic activity. Mice that survive the acute stage of disease develop an immune-mediated demyelinating diseases characterized by viral persistence in white matter tracts and a chronic neuroinflammatory response dominated by T cells and macrophages. Early following JHMV infection, there is a dynamic expression of chemokines and chemokine receptors that contribute to neuroinflammation by regulating innate and adaptive immune responses as well influencing glial biology. In response to JHMV infection, we have shown that signaling through the chemokine receptor CXCR2 contributes to host defense through recruitment of polymorphonuclear cells (PMNs to the CNS that enhance permeability of the blood-brain-barrier (BBB and facilitating entry of virus-specific T cells into the parenchyma. Further, CXCR2 promotes the protection of oligodendroglia from cytokine-induced apoptosis and restricts the severity of demyelination. This review covers aspects related to the role of CXCR2 in host defense and disease in response to JHMV infection.

  17. PET imaging of focal demyelination and remyelination in a rat model of multiple sclerosis: comparison of [11C]MeDAS, [11C]CIC and [11C]PIB.

    Science.gov (United States)

    Faria, Daniele de Paula; Copray, Sjef; Sijbesma, Jurgen W A; Willemsen, Antoon T M; Buchpiguel, Carlos A; Dierckx, Rudi A J O; de Vries, Erik F J

    2014-05-01

    In this study, we compared the ability of [(11)C]CIC, [(11)C]MeDAS and [(11)C]PIB to reveal temporal changes in myelin content in focal lesions in the lysolecithin rat model of multiple sclerosis. Pharmacokinetic modelling was performed to determine the best method to quantify tracer uptake. Sprague-Dawley rats were stereotactically injected with either 1 % lysolecithin or saline into the corpus callosum and striatum of the right brain hemisphere. Dynamic PET imaging with simultaneous arterial blood sampling was performed 7 days after saline injection (control group), 7 days after lysolecithin injection (demyelination group) and 4 weeks after lysolecithin injection (remyelination group). The kinetics of [(11)C]CIC, [(11)C]MeDAS and [(11)C]PIB was best fitted by Logan graphical analysis, suggesting that tracer binding is reversible. Compartment modelling revealed that all tracers were fitted best with the reversible two-tissue compartment model. Tracer uptake and distribution volume in lesions were in agreement with myelin status. However, the slow kinetics and homogeneous brain uptake of [(11)C]CIC make this tracer less suitable for in vivo PET imaging. [(11)C]PIB showed good uptake in the white matter in the cerebrum, but [(11)C]PIB uptake in the cerebellum was low, despite high myelin density in this region. [(11)C]MeDAS distribution correlated well with myelin density in different brain regions. This study showed that PET imaging of demyelination and remyelination processes in focal lesions is feasible. Our comparison of three myelin tracers showed that [(11)C]MeDAS has more favourable properties for quantitative PET imaging of demyelinated and remyelinated lesions throughout the CNS than [(11)C]CIC and [(11)C]PIB.

  18. PET imaging of focal demyelination and remyelination in a rat model of multiple sclerosis: comparison of [{sup 11}C]MeDAS, [{sup 11}C]CIC and [{sup 11}C]PIB

    Energy Technology Data Exchange (ETDEWEB)

    Paula Faria, Daniele de [University of Groningen, University Medical Center Groningen, Department of Nuclear Medicine and Molecular Imaging, Groningen (Netherlands); University of Groningen, University Medical Center Groningen, Department of Neuroscience, Groningen (Netherlands); University of Sao Paulo Medical School, Center of Nuclear Medicine, Sao Paulo (Brazil); Copray, Sjef [University of Groningen, University Medical Center Groningen, Department of Neuroscience, Groningen (Netherlands); Sijbesma, Jurgen W.A.; Willemsen, Antoon T.M.; Dierckx, Rudi A.J.O.; Vries, Erik F.J. de [University of Groningen, University Medical Center Groningen, Department of Nuclear Medicine and Molecular Imaging, Groningen (Netherlands); Buchpiguel, Carlos A. [University of Sao Paulo Medical School, Center of Nuclear Medicine, Sao Paulo (Brazil)

    2014-05-15

    In this study, we compared the ability of [{sup 11}C]CIC, [{sup 11}C]MeDAS and [{sup 11}C]PIB to reveal temporal changes in myelin content in focal lesions in the lysolecithin rat model of multiple sclerosis. Pharmacokinetic modelling was performed to determine the best method to quantify tracer uptake. Sprague-Dawley rats were stereotactically injected with either 1 % lysolecithin or saline into the corpus callosum and striatum of the right brain hemisphere. Dynamic PET imaging with simultaneous arterial blood sampling was performed 7 days after saline injection (control group), 7 days after lysolecithin injection (demyelination group) and 4 weeks after lysolecithin injection (remyelination group). The kinetics of [{sup 11}C]CIC, [{sup 11}C]MeDAS and [{sup 11}C]PIB was best fitted by Logan graphical analysis, suggesting that tracer binding is reversible. Compartment modelling revealed that all tracers were fitted best with the reversible two-tissue compartment model. Tracer uptake and distribution volume in lesions were in agreement with myelin status. However, the slow kinetics and homogeneous brain uptake of [{sup 11}C]CIC make this tracer less suitable for in vivo PET imaging. [{sup 11}C]PIB showed good uptake in the white matter in the cerebrum, but [{sup 11}C]PIB uptake in the cerebellum was low, despite high myelin density in this region. [{sup 11}C]MeDAS distribution correlated well with myelin density in different brain regions. This study showed that PET imaging of demyelination and remyelination processes in focal lesions is feasible. Our comparison of three myelin tracers showed that [{sup 11}C]MeDAS has more favourable properties for quantitative PET imaging of demyelinated and remyelinated lesions throughout the CNS than [{sup 11}C]CIC and [{sup 11}C]PIB. (orig.)

  19. Progression of motor axon dysfunction and ectopic Na(v)1.8 expression in a mouse model of Charcot-Marie-Tooth disease 1B

    DEFF Research Database (Denmark)

    Rosberg, Mette R.; Alvarez Herrero, Susana; Klein, Dennis

    2016-01-01

    . By comparison with WT littermates, P0+/- showed a decreasing motor performance with age. This was associated with a progressive reduction in amplitude and increase in latency of the plantar compound muscle action potential (CMAP) evoked by stimulation of the tibial nerve at ankle. This progressive functional......Mice heterozygously deficient for the myelin protein P0 gene (P0+/-) develop a slowly progressing neuropathy modeling demyelinating Charcot-Marie-Tooth disease (CMT1B). The aim of the study was to investigate the long-term progression of motor dysfunction in P0+/- mice at 3, 7, 12 and 20months...... impairment was in contrast to the mild demyelinating neuropathy of the tibial nerve revealed by histology. "Threshold-tracking" studies showed impaired motor axon excitability in P0+/- from 3months. With time, there was a progressive reduction in threshold deviations during both depolarizing...

  20. Pathophysiology and spectrum of diseases caused by defects in lymphocyte cytotoxicity.

    Science.gov (United States)

    Meeths, Marie; Chiang, Samuel C C; Löfstedt, Alexandra; Müller, Martha-Lena; Tesi, Bianca; Henter, Jan-Inge; Bryceson, Yenan T

    2014-07-01

    In experimental settings, lymphocyte cytotoxicity has been recognized as a central mechanism for immune defense against infected and neoplastic cells. More recently, molecular determinants of lymphocyte cytotoxicity have been identified through studies of rare, inherited hyperinflammatory and lymphoproliferative syndromes that include hemophagocytic lymphohistiocytosis (HLH). These studies have unraveled a set of genes pivotal for the biogenesis and directed release of perforin-containing lysosomes that mediate target cell killing, in addition to other pathways including Fas that also contribute to induction of cell death. Furthermore, studies of such human primary immunodeficiencies have highlighted non-redundant roles of perforin for maintenance of immune homeostasis. Besides providing mechanistic insights to lymphocyte cytotoxicity, studies of individuals with rare hyperinflammatory diseases are highlighting the relevance of lymphocyte cytotoxicity to more common human diseases. It is increasingly recognized that mutations abrogating lymphocyte cytotoxicity not only cause HLH, but also are associated with susceptibility to cancer and autoimmune syndromes. In addition, patients may initially be present with neurological symptoms or severe infectious disease masquerading as variable immunodeficiency syndrome. Here, we highlight new knowledge regarding the molecular mechanisms regulating lymphocyte cytotoxicity and review how mutations in genes associated with HLH cause disease. We also discuss the wider implications of impairments in lymphocyte cytotoxicity for human disease predisposition. Copyright © 2014 Elsevier Inc. All rights reserved.

  1. Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)

    Science.gov (United States)

    ... and abnormal sensations. CIDP is closely related to Guillain-Barre syndrome and it is considered the chronic counterpart ... and abnormal sensations. CIDP is closely related to Guillain-Barre syndrome and it is considered the chronic counterpart ...

  2. MRI EVALUATION OF A CASE OF KRABBE DISEASE

    Directory of Open Access Journals (Sweden)

    Pramod Setty

    2015-03-01

    Full Text Available Krabbe disease, or globoid cell leukodystrophy, is a demyelinating disorder caused by a genetic deficiency of lysosomal enzyme galactocerebrosidase (GALC, a key component in metabolic pathways of myelin turnover and breakdown. The GALC gene maps to chromosome 1 4q24. 3 to 14q32. 1. The most frequent form of Krabbe disease has an infantile onset, whereas the late - onset form is rare. We present the MR imaging findings in a 5 month old male baby presenting with features of spastic paraplegia diagnosed biochemically as Krabbe Disease. Their MR images showed selective, increased signal intensity on T2 - weighted sequences along the corticospinal tracts. Proton MR Spectroscopy showed increased choline and myo - inositol peaks in the affected white matter. The MR imaging pa ttern is characteristic and, in the appropriate clinical setting, may suggest the diagnosis.

  3. MR imaging of metabolic white matter diseases: Therapeutic response

    International Nuclear Information System (INIS)

    Gebarski, S.S.; Allen, R.

    1987-01-01

    In metabolic diseases affecting the brain, MR imaging abnormalities include white-matter signal aberrations suggesting myelination delay, dysmyelination and demyelination, pathologic iron storage, and finally, loss of substance usually in a nonspecific pattern. The authors suggest that MR imaging may have therapeutic implications: (1) classic galactosemia - white-matter signal aberration became normal after dietary therapy; (2) phenylketonuria - age- and sex-matched treated and nontreated adolescents showed marked differences in brain volume, with the treated patient's volume nearly normal; (3) maple syrup urine disease - gross white-matter signal aberration became nearly normal after dietary therapy; and (4) hyperglycinemia - relentless progression of white-matter signal aberration and loss of brain substance despite therapy. These data suggest that brain MR imaging may provide a therapeutic index in certain metabolic diseases

  4. Inhibition of peptidyl-arginine deiminases reverses protein-hypercitrullination and disease in mouse models of multiple sclerosis

    Directory of Open Access Journals (Sweden)

    Mario A. Moscarello

    2013-03-01

    Multiple sclerosis (MS is the most common CNS-demyelinating disease of humans, showing clinical and pathological heterogeneity and a general resistance to therapy. We first discovered that abnormal myelin hypercitrullination, even in normal-appearing white matter, by peptidylarginine deiminases (PADs correlates strongly with disease severity and might have an important role in MS progression. Hypercitrullination is known to promote focal demyelination through reduced myelin compaction. Here we report that 2-chloroacetamidine (2CA, a small-molecule, PAD active-site inhibitor, dramatically attenuates disease at any stage in independent neurodegenerative as well as autoimmune MS mouse models. 2CA reduced PAD activity and protein citrullination to pre-disease status. In the autoimmune models, disease induction uniformly induced spontaneous hypercitrullination with citrulline+ epitopes targeted frequently. 2CA rapidly suppressed T cell autoreactivity, clearing brain and spinal cord infiltrates, through selective removal of newly activated T cells. 2CA essentially prevented disease when administered before disease onset or before autoimmune induction, making hypercitrullination, and specifically PAD enzymes, a therapeutic target in MS models and thus possibly in MS.

  5. Guillain-Barré syndrome, tuberculosis and inflammatory bowel disease: a multiple association

    Directory of Open Access Journals (Sweden)

    de la Torre Ricardo

    2010-07-01

    Full Text Available Abstract Guillain-Barré syndrome (GBS has been associated with both infective or non-infective aetiologies. GBS is usually preceded by acute respiratory or gastrointestinal infection but its association with tuberculosis has been exceptionally reported. Inflammatory bowel disease (IBD is associated with clinical manifestations involving the neurological system, peripheral neuropathy is known to be related to IBD and, either demyelinating or axonal involvement of peripheral nerves have been described. We report an unusual case of GBS associated with lymph node tuberculosis and ulcerative colitis.

  6. Disease: H01719 [KEGG MEDICUS

    Lifescience Database Archive (English)

    Full Text Available sease include demyelinating, inflammatory, toxic, nutritional, compressive, infiltrative, hereditary, trauma...09] Linezolid [DR:D00947] Amiodarone [DR:D00636] ... Corticosteroids Mecobalamin [DR:D03246] (for nutrition

  7. Atypical presentation of probable Creutzfeldt-Jakob disease associated with anti-Zic4 antibody: Literature review of neuronal antibodies in Creutzfeldt-Jakob disease.

    Science.gov (United States)

    Salazar, Richard

    2018-05-01

    Sporadic Creutzfeldt-Jakob disease is a prion disease characterized by rapidly progressive dementia, ataxia and myoclonus. Atypical phenotype masquerading as stroke, movement disorders or autoimmune encephalitis have been described. Here, I report a probable case of sCJD with an atypical presentation associated with anti-Zic4 antibody and review the literature of neuronal antibodies in CJD. A 70 year-old gentleman is admitted with a 2-month history of recurrent stroke-like symptoms associated with behavioral disturbances, gait ataxia and rapidly progressive dementia. His initial examination demonstrated akinetic mutism, diffuse rigidity, dysautononia, and Cheyne-Stokes respiration. Over the following weeks his condition progressed to profound coma. A comprehensive infectious, metabolic, inflammatory and autoimmune work-up yielded negative results. Empiric immunosuppressive therapy ensued. He expired three months after symptoms onset. Autopsy was not performed. After his demise, prion tests came back abnormal for elevated 14-3-3 protein, total tau and positive RTQuIC. Later on, anti-Zic4 antibodies were found in serum. This case underscores the importance of a high index of suspicion for CJD even in case of atypical features or the concurrence of neuronal antibodies. Further larger prospective studies on the prevalence of these neuronal antibodies in CJD and the contribution of these autoantibodies to disease pathophysiology are necessary. Copyright © 2018 Elsevier B.V. All rights reserved.

  8. Subcutaneous immunoglobulin for maintenance treatment in chronic inflammatory demyelinating polyneuropathy (PATH): a randomised, double-blind, placebo-controlled, phase 3 trial.

    Science.gov (United States)

    van Schaik, Ivo N; Bril, Vera; van Geloven, Nan; Hartung, Hans-Peter; Lewis, Richard A; Sobue, Gen; Lawo, John-Philip; Praus, Michaela; Mielke, Orell; Durn, Billie L; Cornblath, David R; Merkies, Ingemar S J

    2018-01-01

    Approximately two-thirds of patients with chronic inflammatory demyelinating polyneuropathy (CIDP) need long-term intravenous immunoglobulin. Subcutaneous immunoglobulin (SCIg) is an alternative option for immunoglobulin delivery, but has not previously been investigated in a large trial of CIDP. The PATH study compared relapse rates in patients given SCIg versus placebo. Between March 12, 2012, and Sept 20, 2016, we studied patients from 69 neuromuscular centres in North America, Europe, Israel, Australia, and Japan. Adults with definite or probable CIDP who responded to intravenous immunoglobulin treatment were eligible. We randomly allocated participants to 0·2 g/kg or 0·4 g/kg of a 20% SCIg solution (IgPro20) weekly versus placebo (2% human albumin solution) for maintenance treatment for 24 weeks. We did randomisation in a 1:1:1 ratio with an interactive voice and web response system with a block size of six, stratified by region (Japan or non-Japan). The primary outcome was the proportion of patients with a CIDP relapse or who were withdrawn for any other reason during 24 weeks of treatment. Patients, caregivers, and study personnel, including those assessing outcomes, were masked to treatment assignment. Analyses were done in the intention-to-treat and per-protocol sets. This trial is registered with ClinicalTrials.gov, number NCT01545076. In this randomised, double-blind, placebo-controlled trial, we randomly allocated 172 patients: 57 (33%) to the placebo group, 57 (33%) to the low-dose group, and 58 (34%) to the high-dose group. In the intention-to-treat set, 36 (63% [95% CI 50-74]) patients on placebo, 22 (39% [27-52]) on low-dose SCIg, and 19 (33% [22-46]) on high-dose SCIg had a relapse or were withdrawn from the study for other reasons (p=0·0007). Absolute risk reductions were 25% (95% CI 6-41) for low-dose versus placebo (p=0·007), 30% (12-46) for high-dose versus placebo (p=0·001), and 6% (-11 to 23) for high-dose versus low-dose (p=0·32

  9. Clinico-Electrophysiological and Genetic Overlaps and Magnetic Resonance Imaging Findings in Charcot-Marie- Tooth Disease: A Pilot Study from Western India.

    Science.gov (United States)

    Khadilkar, Satish Vasant; Patil, Nahush D; Kadam, Nikhil Dhananjay; Mansukhani, Khushnuma A; Patel, Bhagyadhan A

    2017-01-01

    Charcot-Marie-Tooth (CMT) disease is clinically and genetically heterogeneous. There are no published series describing clinical, electrophysiological, and genetic information on CMT from the Indian subcontinent. Magnetic resonance imaging (MRI) neurography technique provides useful information about the plexus and roots and can be employed in patients with CMT. A prospective, observational study carried out at a tertiary care hospital in Western India. CMT patients fulfilling the UK Genetic Testing Network criteria were included. They underwent clinical, electrophysiological, radiological, and multigene panel testing. Totally 22 patients (19 males, 3 females; 18 sporadic and 4 familial cases) were studied. Pes cavus (19), hammer toes (16), and scoliosis was seen in 1 patient. Electrophysiology revealed motor predominant neuropathy with 15 demyelinating (10 uniform and 5 multifocal) and 7 axonal patterns. Thickened lumbosacral plexuses on MRI neurography were evident in 6/10 studied patients, all 6 having demyelinating neuropathy. Genetic analysis identified PMP22, GJB1, SH3TC2, HSPB1, SPTLC2, MPZ, AARS, and NEFH gene mutations. This small series documents the pattern of CMT neuropathies as seen in Western India. Clinico-electrophysiological and genetic diagnosis showed general concordance some overlaps and reiterated advantages of gene panel testing in this heterogeneous group of neuropathies. MRI neurography was useful as an additional investigation to detect nerve enlargement in patients with demyelinating neuropathies.

  10. Splenial lesions of the corpus callosum: Disease Spectrum and MRI findings

    Energy Technology Data Exchange (ETDEWEB)

    Park, Sung Eun; Choi, Dae Seob; Shin, Hwa Seon; Baek, Hye Jin; Choi, Ho Cheol; Kim, Ji Eun; Choi, Hye Young; Park, Min Jung [Dept. of Radiology, Gyeongsang National University School of Medicine, Jinju (Korea, Republic of)

    2017-08-01

    The corpus callosum (CC) is the largest white matter structure in the brain, consisting of more than 200–250 million axons that provide a large connection mainly between homologous cerebral cortical areas in mirror image sites. The posterior end of the CC is the thickest part, which is called the slenium. Various diseases including congenital to acquired lesions including congenital anomalies, traumatic lesions, ischemic diseases, tumors, metabolic, toxic, degenerative, and demyelinating diseases, can involve the splenium of the CC and their clinical symptoms and signs are also variable. Therefore, knowledge of the disease entities and the imaging findings of lesions involving the splenium is valuable in clinical practice. MR imaging is useful for the detection and differential diagnosis of splenial lesions of the CC. In this study, we classify the disease entities and describe imaging findings of lesions involving the splenium of the CC based on our experiences and a review of the literature.

  11. De novo PMP2 mutations in families with type 1 Charcot-Marie-Tooth disease.

    Science.gov (United States)

    Motley, William W; Palaima, Paulius; Yum, Sabrina W; Gonzalez, Michael A; Tao, Feifei; Wanschitz, Julia V; Strickland, Alleene V; Löscher, Wolfgang N; De Vriendt, Els; Koppi, Stefan; Medne, Livija; Janecke, Andreas R; Jordanova, Albena; Zuchner, Stephan; Scherer, Steven S

    2016-06-01

    We performed whole exome sequencing on a patient with Charcot-Marie-Tooth disease type 1 and identified a de novo mutation in PMP2, the gene that encodes the myelin P2 protein. This mutation (p.Ile52Thr) was passed from the proband to his one affected son, and segregates with clinical and electrophysiological evidence of demyelinating neuropathy. We then screened a cohort of 136 European probands with uncharacterized genetic cause of Charcot-Marie-Tooth disease and identified another family with Charcot-Marie-Tooth disease type 1 that has a mutation affecting an adjacent amino acid (p.Thr51Pro), which segregates with disease. Our genetic and clinical findings in these kindred demonstrate that dominant PMP2 mutations cause Charcot-Marie-Tooth disease type 1. © The Author (2016). Published by Oxford University Press on behalf of the Guarantors of Brain. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

  12. The frequencies of Killer immunoglobulin-like receptors and their HLA ligands in chronic inflammatory demyelinating polyradiculoneuropathy are similar to those in Guillian Barre syndrome but differ from those of controls, suggesting a role for NK cells in pathogenesis.

    Science.gov (United States)

    Blum, Stefan; Csurhes, Peter; McCombe, Pamela

    2015-08-15

    Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an acquired inflammatory neuropathy, which has similar clinical and pathological features to Guillain-Barré Syndrome (GBS), but differs in time course. We investigated the frequency of genes encoding Killer immunoglobulin-like receptors and their HLA ligands in subjects with CIDP, in subjects with GBS and in healthy controls. There were no differences in KIR gene frequency among the 3 groups. The gene frequencies for HLA-B Bw4-I were significantly greater in CIDP than HC, but did not differ from GBS. The frequency of the combination of 3DL1/HLA-B Bw4I was greater in CIDP than HC, but did not differ from that of GBS. These data raise the possibility of NK cell function being an important factor in the pathogenesis of CIDP. Copyright © 2015 Elsevier B.V. All rights reserved.

  13. MARCHIAFAVA-BIGNAMI DISEASE (MBD AND DIFFUSION TENSOR IMAGE (DTI TRACTOGRAPHY

    Directory of Open Access Journals (Sweden)

    Priscilla Chukwueke

    2015-06-01

    Full Text Available Marchiafava-Bignami Disease (MBD is a rare central nervous system (CNS disease characterized by demyelination of the corpus callosum. It is mostly found in men with alcohol use disorder and malnutrition with cases reported worldwide across all races. The onset of the disease may be sudden presenting with stupor, coma or seizures while some may present with gait abnormality (spasticity, psychiatric problems, hemiparesis, aphasia, apraxia and incontinence with a resultant high morbidity and mortality rates. Case description: patient is a 30 year old left handed African-American, who presented with c/o altered mental status, urinary incontinence, slurred speech and left-sided weakness. The diagnosis of MBD was confirmed with DTI Tractography which showed significantly diminished commissural fibers extending to the right central semiovale lesion, near absent or significantly diminished commissural fiber extending through the corpus callosum indicating demyelination. Discussion: MBD is often an incidental diagnosis with high morbidity and mortality. This is different from previous casas because of earlier onset as opposed to onset around age 45, rapid recovery and minimal disability as he could walk independently before discharge from hospital. This case also shows added benefit of the DTI tractography in the diagnosis of MBD.

  14. Proton density differences in signal characteristics of multiple sclerosis plaques versus white matter lesions of small vessel disease and vasculitis on high-field strength MR images

    International Nuclear Information System (INIS)

    Peyster, R.G.; Siegal, T.L.

    1990-01-01

    This paper determines if variations in signal intensity characteristics on multi-spin-echo images obtained with a high-field-strength magnet can be useful in differentiating demyelinating plaques of multiple sclerosis from other pathologic white matter processes due to small vessel disease and vasculities. Using the first of two multi-spin-echo images obtained with a General Electric 1.5-T magnet, the investigators compared signal intensity characteristics in 30 patients with a firm clinical diagnosis of multiple sclerosis versus a control group of 30 patients with a known clinical history of small-vessel disease and vasculitis are isodense to gray matter on proton-density images

  15. POEMS syndrome associated with Castleman disease: a case report and literature review

    Directory of Open Access Journals (Sweden)

    Juan Kang

    2014-06-01

    Full Text Available Polyneuropathy, organomegaly, endocrinopathy, M proteins, and skin changes (POEMS syndrome is a multisystemic disorder that clinically manifests as paraneoplastic and monoclonal plasma cell dyscrasia. Its acronym is derived from its principal characteristics: polyneuropathy, organomegaly, endocrinopathy, M proteins, and skin changes. Here, the authors reported a case of POEMS syndrome that was also associated with Castleman disease. A 53-year-old female patient was admitted to our hospital with limb weakness, numbness, edema, abdominal distention, and fever. Physical examination revealed tetraplegia, paraesthesia, and hyporeflexia in all four limbs, in addition to lymphadenectasis, splenomegaly, skin hyperpigmentation, hypertrichosis, and pitting edema. Laboratory tests and imaging revealed thrombocytosis, hypothyroidism, diabetes, hydropericardium, hydrothorax, splenomegaly, and lymphadenectasis. Electromyography showed the characteristic patterns of both demyelinating disease and axonal degeneration. Serum protein electrophoresis revealed monoclonal immunoglobulin G-lambda paraproteins. Histological examination clearly diagnosed the disease as the hyaline vascular subtype. The final diagnosis in this case was POEMS syndrome in association with Castleman disease.

  16. A minimal unified model of disease trajectories captures hallmarks of multiple sclerosis

    KAUST Repository

    Kannan, Venkateshan

    2017-03-29

    Multiple Sclerosis (MS) is an autoimmune disease targeting the central nervous system (CNS) causing demyelination and neurodegeneration leading to accumulation of neurological disability. Here we present a minimal, computational model involving the immune system and CNS that generates the principal subtypes of the disease observed in patients. The model captures several key features of MS, especially those that distinguish the chronic progressive phase from that of the relapse-remitting. In addition, a rare subtype of the disease, progressive relapsing MS naturally emerges from the model. The model posits the existence of two key thresholds, one in the immune system and the other in the CNS, that separate dynamically distinct behavior of the model. Exploring the two-dimensional space of these thresholds, we obtain multiple phases of disease evolution and these shows greater variation than the clinical classification of MS, thus capturing the heterogeneity that is manifested in patients.

  17. Proton MR spectroscopy in three children with Tay-Sachs disease

    Energy Technology Data Exchange (ETDEWEB)

    Aydin, Kubilay; Bakir, Baris; Terzibasioglu, Ege [Istanbul University, Neuroradiology Division, Department of Radiology, Istanbul (Turkey); Tatli, Burak; Ozmen, Meral [Istanbul University, Department of Paediatric Neurology, Istanbul (Turkey)

    2005-11-01

    Tay-Sachs disease is an inherited metabolic disease caused by the accumulation of GM{sub 2} gangliosides in the central nervous system. Deficiency of hexosaminidase A leads to the accumulation of gangliosides in neurons, axons and glial cells. To present the cranial MRI and proton MR spectroscopy findings of children of Tay-Sachs disease. Three children aged 10, 20 and 21 months were examined. On T2-weighted MR images there were hyperintense signal changes in the basal ganglia and cerebral white matter. MR spectroscopy demonstrated an increase in myoinositol/creatine and choline/creatine ratios with a decrease in the N-acetyl aspartate/creatine ratio. The spectroscopy findings support demyelination, gliosis and neuronal loss in the neuropathological process of Tay-Sachs disease. (orig.)

  18. A novel mutation in ABCA1 gene causing Tangier Disease in an Italian family with uncommon neurological presentation

    Directory of Open Access Journals (Sweden)

    Marco Ceccanti

    2016-11-01

    Full Text Available Tangier disease is an autosomal recessive disorder characterized by severe reduction in HDL-cholesterol and peripheral lipid storage. We describe a family with c.5094C>A p.Tyr16980* mutation in the ABCA1 gene, clinically characterized by syringomyelic-like anesthesia, demyelinating multineuropathy and reduction in intraepidermal small fibers innervation. In the proband patient, cardiac involvement determined a myocardial infarction; lipid storage was demonstrated in gut, cornea and aortic wall. The reported ABCA1 mutation has never been described before in a Tangier family.

  19. Unilateral oculomotor palsy in Charcot-Marie-Tooth disease 1A (CMT 1A).

    Science.gov (United States)

    Posa, A; Emmer, A; Kornhuber, M E

    2017-04-01

    Charcot-Marie-Tooth disease (CMT) type 1A is the most common form of CMT 1 and one of the autosomal dominant demyelinating hereditary motor and sensory neuropathies (HMSN). Cranial nerves may be frequently subclinically affected in CMT disease. However manifest clinical signs of cranial nerve involvement are rare. This case comprise neurological, ophthalmological, internal medicine and ear-nose-throat investigation, motor and sensory nerve conduction velocity, auditory evoked potentials and orbicularis-oculi reflex measurements, lumbar puncture and blood examination, inclusive molecular genetic testing, as well as electrocardiogram and cranial imaging such as computer tomography and magnetic resonance imaging RESULTS: The present case shows a Charcot-Marie-Tooth (CMT) 1A patient with complete unilateral oculomotor palsy in combination with predominant ipsilateral subclinical trigeminal demyelination. A combined of third and fifth cranial nerves as in our patient has not been reported yet. This case shows cranial nerve involvement as an unusual leading symptom of CMT 1A. It may remind us that hereditary neuropathies have to be taken into consideration in patients with slowly progressing unilateral or asymmetric cranial neuropathies. Copyright © 2017 Elsevier B.V. All rights reserved.

  20. Adult cerebral adrenoleukodystrophy and Addison's disease in a female carrier.

    Science.gov (United States)

    Chen, Xiaoyan; Chen, Zhiye; Huang, Dehui; Liu, Xiaofeng; Gui, Qiuping; Yu, Shengyuan

    2014-07-10

    We described a 38-year-old woman of rapidly progressive dementia with white matter encephalopathy and death. She had Addison's disease but the adrenal glands were hyperplastic. Brain magnetic resonance imaging revealed diffuse white matter lesion predominantly in the frontal lobe with band-like contrast enhancement. l-Methyl-11C-methionine positron emission tomography revealed accumulation of tracer in bilateral frontal lobes. Stereotactic biopsy demonstrated demyelination changes. A number of urinary organic acids were elevated. Adrenoleukodystrophy was diagnosed by elevated plasma very long chain fatty acid and ABCD1 gene mutation (C1544C/T). Adrenoleukodystrophy should be considered as a differential diagnosis in women with rapidly progressive white matter encephalopathy. Copyright © 2014 Elsevier B.V. All rights reserved.

  1. Chronic inflammatory demyelinating polyradiculoneuropathy: two cases with cervical spinal cord compression Polirradiculoneuropatia desmielinizante inflamatória crônica: dois casos com síndrome de compressão medular

    Directory of Open Access Journals (Sweden)

    Marcos R.G. de Freitas

    2005-09-01

    Full Text Available Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP is a peripheral nerve disorder probably due to an immunological disturb. It evolves either in a steadily progressive or in a relapsing and fluctuating course. Weakness is mainly in the lower limbs proximally and distally. The electromyography is demyelinating. The cerebral spinal fluid protein is most of times elevated. Sometimes enlarged nerves are found. There are few cases described with spinal cord compression due to hypertrophic spinal nerve roots. Two patients (females, 66 and 67 years old with diagnosis of a long standing CIDP are described. In the first one, the evolution was characterized by remission and relapsing course. The second patient had a chronic and progressive course. These patients presented after a long evolution a cervical spinal cord compression syndrome due to hypertrophic cervical roots. Neurologists must be aware of the possibility of development of spinal cord compression by enlarged spinal roots in patients with a long standing CIDP.A polirradiculoneuropatia desmielinizante inflamatória crônica (PDIC é uma afecção dos nervos periféricos de natureza autoimune, com evolução por surtos de exacerbação e remissão ou de evolver progressivo. O acometimento motor é predominante, com fraqueza proximal e distal nos membros inferiores. A eletroneuromiografia é do tipo desmielinizante com bloqueio de condução nervosa em dois ou mais nervos. Há aumento de proteínas do líquor. Com a evolução da doença pode haver espessamento dos nervos distal e/ou proximalmente. Excepcionalmente ocorre compressão da medula espinhal em qualquer segmento por raízes próximas hipertrofiadas. Foram estudadas duas mulheres de 66 e 67 anos respectivamente com quadro de PDIC de longa evolução. A primeira tinha evolução por surtos e na segunda o evolver era progressivo. Nos dois casos o espessamento proximal dos nervos provocou síndrome de compressão medular alta

  2. Single nucleotide polymorphisms in multiple sclerosis: disease susceptibility and treatment response biomarkers.

    Science.gov (United States)

    Pravica, Vera; Popadic, Dusan; Savic, Emina; Markovic, Milos; Drulovic, Jelena; Mostarica-Stojkovic, Marija

    2012-04-01

    Multiple sclerosis (MS) is a chronic inflammatory demyelinating and neurodegenerative disease of the central nervous system characterized by unpredictable and variable clinical course. Etiology of MS involves both genetic and environmental factors. New technologies identified genetic polymorphisms associated with MS susceptibility among which immunologically relevant genes are significantly overrepresented. Although individual genes contribute only a small part to MS susceptibility, they might be used as biomarkers, thus helping to identify accurate diagnosis, predict clinical disease course and response to therapy. This review focuses on recent progress in research on MS genetics with special emphasis on the possibility to use single nucleotide polymorphism of candidate genes as biomarkers of susceptibility to disease and response to therapy.

  3. Receptor alterations associated with spinal motoneuron degeneration in bovine Akabane disease.

    Science.gov (United States)

    Gundlach, A L; Grabara, C S; Johnston, G A; Harper, P A

    1990-05-01

    Akabane disease in cattle is characterized by congenital abnormalities including arthrogryposis, which is characterized by a depletion of spinal ventral horn motoneurons, a loss of axons, and depletion of myelin in the lateral and ventral tracts. These neuropathological changes produced major reductions (70-80%) in the density of muscarinic cholinergic, glycine/strychnine, and central-type benzodiazepine receptors in the ventral horn motor nuclei. The density of peripheral-type benzodiazepine receptors and adenosine A1 receptors was dramatically increased (250-300%) in the lateral and ventral spinal columns, reflecting the proliferation of glial cells. Bovine Akabane disease represents a useful model for assessing the processes and consequences of neuronal degeneration and demyelination and has implications for human diseases such as amyotrophic lateral sclerosis.

  4. Graves' Disease

    Science.gov (United States)

    ... Navigation Endocrine Diseases Acromegaly Adrenal Insufficiency & Addison's Disease Cushing's Syndrome Graves' Disease Hashimoto's Disease Hyperthyroidism (Overactive Thyroid) Hypothyroidism (Underactive Thyroid) ...

  5. Conduction block and tonic pupils in Charcot-Marie-Tooth disease caused by a myelin protein zero p.Ile112Thr mutation.

    LENUS (Irish Health Repository)

    Murphy, Sinéad M

    2011-03-01

    We report a patient with Charcot-Marie-Tooth disease (CMT) due to the p.Ile112Thr mutation in myelin protein zero (MPZ) who presented with a patchy neuropathy with conduction block and tonic pupils. Conduction block is unusual in inherited neuropathies, while pupil abnormalities are recognised to occur in CMT especially due to MPZ mutations. This case highlights that patchy demyelinating neuropathy with conduction block may occur in p.Ile112Thr MPZ mutations. Involvement of the pupils, as in this case, may be a pointer towards a genetic rather than inflammatory cause of neuropathy.

  6. Disease Course and Treatment Responses in Children With Relapsing Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease.

    Science.gov (United States)

    Hacohen, Yael; Wong, Yu Yi; Lechner, Christian; Jurynczyk, Maciej; Wright, Sukhvir; Konuskan, Bahadir; Kalser, Judith; Poulat, Anne Lise; Maurey, Helene; Ganelin-Cohen, Esther; Wassmer, Evangeline; Hemingway, Chery; Forsyth, Rob; Hennes, Eva Maria; Leite, M Isabel; Ciccarelli, Olga; Anlar, Banu; Hintzen, Rogier; Marignier, Romain; Palace, Jacqueline; Baumann, Matthias; Rostásy, Kevin; Neuteboom, Rinze; Deiva, Kumaran; Lim, Ming

    2018-04-01

    Myelin oligodendrocyte glycoprotein antibodies (MOG-Abs) are consistently identified in a range of demyelinating disorders in adults and children. Current therapeutic strategies are largely center specific, and no treatments have been formally evaluated. To examine the clinical phenotypes, treatment responses, and outcomes of children with relapsing MOG-Ab-associated disease. This study prospectively collected demographic, clinical, and radiologic data from 102 patients from 8 countries of the EU Paediatric Demyelinating Disease Consortium from January 1, 2014, through December 31, 2016. Patients were treated according to local protocols. Annualized relapse rates (ARRs) and Expanded Disability Status Scale (EDSS) scores before and during treatment with disease-modifying drugs (DMDs). A total of 102 children were identified (median [range] age, 7.0 [1.5-7.9] years; male to female ratio, 1.0:1.8; white to other race/ethnicity ratio, 3.6:1.0). Original diagnoses were neuromyelitis optica spectrum disorder (44 patients [43.1%]), acute disseminated encephalomyelitis followed by optic neuritis (20 [19.6%]), multiphasic disseminated encephalomyelitis (20 [19.6%]), and relapsing optic neuritis (18 [17.6%]). In all, 464 demyelinating events were reported. Treated patients had more relapses (median, 3.0; range, 1.0-17.0) than untreated patients (median, 1.0; range 1.0-7.0) (P = .009) and higher EDSS scores (median, 1.5; interquartile range, 0-2.5) than untreated patients (median, 1.0; interquartile range, 0-1.5) (P < .001). Fifty-two children (51.0%) received DMDs: 28 (53.8%) were treated with 1 DMD, 17 (32.7%) with 2, and 7 (13.5%) with 3 or more sequential DMDs. Patients relapsed during all treatments, with a total of 127 relapses on treatment reported. No changes in median ARR and EDSS score were observed between the preinitiation and postinitiation phases of interferon beta and glatiramer acetate treatment (n = 11). The median ARR was reduced from 1.84 to 1

  7. Colony-stimulating factor-1 mediates macrophage-related neural damage in a model for Charcot–Marie–Tooth disease type 1X

    Science.gov (United States)

    Groh, Janos; Weis, Joachim; Zieger, Hanna; Stanley, E. Richard; Heuer, Heike

    2012-01-01

    Previous studies in our laboratory have shown that in models for three distinct forms of the inherited and incurable nerve disorder, Charcot–Marie–Tooth neuropathy, low-grade inflammation implicating phagocytosing macrophages mediates demyelination and perturbation of axons. In the present study, we focus on colony-stimulating factor-1, a cytokine implicated in macrophage differentiation, activation and proliferation and fostering neural damage in a model for Charcot–Marie–Tooth neuropathy 1B. By crossbreeding a model for the X-linked form of Charcot–Marie–Tooth neuropathy with osteopetrotic mice, a spontaneous null mutant for colony-stimulating factor-1, we demonstrate a robust and persistent amelioration of demyelination and axon perturbation. Furthermore, functionally important domains of the peripheral nervous system, such as juxtaparanodes and presynaptic terminals, were preserved in the absence of colony-stimulating factor-1-dependent macrophage activation. As opposed to other Schwann cell-derived cytokines, colony-stimulating factor-1 is expressed by endoneurial fibroblasts, as revealed by in situ hybridization, immunocytochemistry and detection of β-galactosidase expression driven by the colony-stimulating factor-1 promoter. By both light and electron microscopic studies, we detected extended cell–cell contacts between the colony-stimulating factor-1-expressing fibroblasts and endoneurial macrophages as a putative prerequisite for the effective and constant activation of macrophages by fibroblasts in the chronically diseased nerve. Interestingly, in human biopsies from patients with Charcot–Marie–Tooth type 1, we also found frequent cell–cell contacts between macrophages and endoneurial fibroblasts and identified the latter as main source for colony-stimulating factor-1. Therefore, our study provides strong evidence for a similarly pathogenic role of colony-stimulating factor-1 in genetically mediated demyelination in mice and Charcot

  8. Colony-stimulating factor-1 mediates macrophage-related neural damage in a model for Charcot-Marie-Tooth disease type 1X.

    Science.gov (United States)

    Groh, Janos; Weis, Joachim; Zieger, Hanna; Stanley, E Richard; Heuer, Heike; Martini, Rudolf

    2012-01-01

    Previous studies in our laboratory have shown that in models for three distinct forms of the inherited and incurable nerve disorder, Charcot-Marie-Tooth neuropathy, low-grade inflammation implicating phagocytosing macrophages mediates demyelination and perturbation of axons. In the present study, we focus on colony-stimulating factor-1, a cytokine implicated in macrophage differentiation, activation and proliferation and fostering neural damage in a model for Charcot-Marie-Tooth neuropathy 1B. By crossbreeding a model for the X-linked form of Charcot-Marie-Tooth neuropathy with osteopetrotic mice, a spontaneous null mutant for colony-stimulating factor-1, we demonstrate a robust and persistent amelioration of demyelination and axon perturbation. Furthermore, functionally important domains of the peripheral nervous system, such as juxtaparanodes and presynaptic terminals, were preserved in the absence of colony-stimulating factor-1-dependent macrophage activation. As opposed to other Schwann cell-derived cytokines, colony-stimulating factor-1 is expressed by endoneurial fibroblasts, as revealed by in situ hybridization, immunocytochemistry and detection of β-galactosidase expression driven by the colony-stimulating factor-1 promoter. By both light and electron microscopic studies, we detected extended cell-cell contacts between the colony-stimulating factor-1-expressing fibroblasts and endoneurial macrophages as a putative prerequisite for the effective and constant activation of macrophages by fibroblasts in the chronically diseased nerve. Interestingly, in human biopsies from patients with Charcot-Marie-Tooth type 1, we also found frequent cell-cell contacts between macrophages and endoneurial fibroblasts and identified the latter as main source for colony-stimulating factor-1. Therefore, our study provides strong evidence for a similarly pathogenic role of colony-stimulating factor-1 in genetically mediated demyelination in mice and Charcot-Marie-Tooth type 1

  9. Crohn's Disease

    Science.gov (United States)

    ... Kidney Disease Weight Management Liver Disease Urologic Diseases Endocrine Diseases Diet & Nutrition Blood Diseases Diagnostic Tests La información ... Kidney Disease Weight Management Liver Disease Urologic Diseases Endocrine Diseases Diet & Nutrition Blood Diseases Diagnostic Tests La información ...

  10. Assessment of demyelination, edema, and gliosis by in vivo determination of T1 and T2 in the brain of patients with acute attack of multiple sclerosis

    DEFF Research Database (Denmark)

    Larsson, H B; Frederiksen, J; Petersen, J

    1989-01-01

    This study intended to investigate the possibility of magnetic resonance (MR) to characterize the acute plaque due to multiple sclerosis (MS). To obtain information, in vivo measurements of relaxation processes were performed in 10 patients with known acute MS plaques, using a whole-body supercon......This study intended to investigate the possibility of magnetic resonance (MR) to characterize the acute plaque due to multiple sclerosis (MS). To obtain information, in vivo measurements of relaxation processes were performed in 10 patients with known acute MS plaques, using a whole......-body superconductive MR-scanner, operating at 1.5 T. The measurements were repeated several times, from onset of the disease and during remission by use of six-point partial saturation inversion recovery and 32-echo multiple spin-echo sequences, giving T1 and T2, respectively. We also focused on the issue, whether T1...

  11. Melatonin attenuates scopolamine-induced cognitive impairment via protecting against demyelination through BDNF-TrkB signaling in the mouse dentate gyrus.

    Science.gov (United States)

    Chen, Bai Hui; Park, Joon Ha; Lee, Tae-Kyeong; Song, Minah; Kim, Hyunjung; Lee, Jae Chul; Kim, Young-Myeong; Lee, Choong-Hyun; Hwang, In Koo; Kang, Il Jun; Yan, Bing Chun; Won, Moo-Ho; Ahn, Ji Hyeon

    2018-04-01

    Animal models of scopolamine-induced amnesia are widely used to study underlying mechanisms and treatment of cognitive impairment in neurodegenerative diseases such as Alzheimer's disease (AD). Previous studies have identified that melatonin improves cognitive dysfunction in animal models. In this study, using a mouse model of scopolamine-induced amnesia, we assessed spatial and short-term memory functions for 4 weeks, investigated the expression of myelin-basic protein (MBP) in the dentate gyrus, and examined whether melatonin and scopolamine cotreatment could keep cognitive function and MBP expression. In addition, to study functions of melatonin for keeping cognitive function and MBP expression, we examined expressions of brain-derived neurotrophic factor (BDNF) and tropomycin receptor kinase B (TrkB) in the mouse dentate gyrus. Scopolamine (1 mg/kg) and melatonin (10 mg/kg) were intraperitoneally treated for 2 and 4 weeks. Two and 4 weeks after scopolamine treatment, mice showed significant cognitive impairment; however, melatonin and scopolamine cotreatment recovered cognitive impairment. Two and 4 weeks of scopolamine treatment, the density of MBP immunoreactive myelinated nerve fibers was significantly decreased in the dentate gyrus; however, scopolamine and melatonin cotreatment significantly increased the scopolamine-induced reduction of MBP expression in the dentate gyrus. Furthermore, the cotreatment of scopolamine and melatonin significantly increased the scopolamine-induced decrease of BDNF and TrKB immunoreactivity in the dentate gyrus. Taken together, our results indicate that melatonin treatment exerts anti-amnesic effect and restores the scopolamine-induced reduction of MBP expression through increasing BDNF and TrkB expressions in the mouse dentate gyrus. Copyright © 2018 Elsevier B.V. All rights reserved.

  12. Periapical Infection Masquerading as a Nasal Pustule

    African Journals Online (AJOL)

    alveolar bone.[2] With the combined host and bacterial factors activities, the inflammatory and immunological processes will induce bone resorption, resulting in the formation of an intraoral or extraoral sinus.[4] When extraoral .... healing of the sinus in the floor of the right nostril. Canal was obturated with guttapercha using ...

  13. Diaphragmatic Hernia Masquerading as Pleural Effusion

    African Journals Online (AJOL)

    Diaphragmatic injuries result from either blunt or penetrating trauma. They are usually associated with thoraco-abdominal trauma, but may rarely occur in isolation. Acute traumatic rupture of the diaphragm may go unnoticed and there may be a delay between the injury and diagnosis. As many as 30% of diaphragmatic ...

  14. National ornament and the imperial masquerade

    Directory of Open Access Journals (Sweden)

    Nóra Veszprémi

    2015-12-01

    Full Text Available Review of Rebecca Houze, Textiles, Fashion, and Design Reform in Austria–Hungary before the First World War: Principles of Dress: Rebecca Houze’s book is a thoroughly researched and original study of the impact of design reform on textile production and fashion in Austria-Hungary in the late nineteenth and early twentieth century which centres its argument around the Bekleidungsprinzip (“principle of dress” put forward by Gottfried Semper. It discusses the role of newly founded applied arts museums, industrial exhibitions, the concept of “house industry,” and the upsurge of interest in folk crafts in this process, while also placing a strong emphasis on the role of women as producers of textiles. One of the main virtues of the book is its wide scope which manages to investigate endeavours informed by different and sometimes opposed national and imperial interests in their complicated interconnectedness. It is a pity that this broad perspective is narrowed down in the second part of the book which focuses on with turn-of-the-century Vienna and does not deal with modernist tendencies in Hungarian design. Nevertheless, this part of the book also provides a fascinating discussion of its own subject, and the monograph as a whole is a valuable contribution to its field of study.

  15. Intrathoracic lipoma masquerading as subclavian artery trauma.

    LENUS (Irish Health Repository)

    Munro, P T

    2012-02-03

    A 58 year old man was admitted to the accident and emergency department following an industrial accident in which he sustained a three part fracture dislocation of his right humerus. Chest radiography revealed a large mass in the right upper hemithorax and, when the patient became hypotensive, an emergency thoracotomy was performed. The mass was found to be a massive intrathoracic lipoma. This case shows how preexisting intrathoracic lesions may be mistaken for subclavian or great vessel trauma following violent shoulder girdle injury. The differential diagnosis of traumatic and non-traumatic intrathoracic mass lesions in chest radiography should be considered carefully.

  16. Periduodenal Tuberculosis masquerading as Annular Pancreas ...

    African Journals Online (AJOL)

    Gastrointestinal tuberculosis is common in Africa. Nonetheless, isolated duodenal involvement is rare, and is more likely to mimic other causes of duodenal obstruction. We report a patient who succumbed to an isolated mid duodenal tuberculosis, diagnosed at laparatomy, whose clinical presentation, endoscopy and ...

  17. Masquerading Mycobacterium: Rectal Growth or Tuberculosis ...

    African Journals Online (AJOL)

    ABSTRACT: A 37-year old male presented to us with history of lower abdominal pain for 6 months. His physical examination revealed a rectal mass of approximately 1centimeter. He was investigated for possible rectal growth with sigmoidoscopy and biopsy. The histopathological examination (HPE) showed a non-specific ...

  18. Infantile Hemangiomas Masquerading as Other Periocular Disorders

    Directory of Open Access Journals (Sweden)

    Jennifer Hsu

    2012-01-01

    Full Text Available Periocular tumors are common in infancy. The most common periocular tumors are capillary hemangiomas, which are present in 1-2% of newborns and develop in 10%–12% of children by the age of 1 year old. Deep capillary hemangiomas may be more challenging to diagnose than superficial capillary hemangiomas and can be confused with other orbital lesions. Deep orbital hemangiomas can mimic teratoma, lymphangioma, rhabdomyosarcoma, metastatic neuroblastoma, and granulocytic sarcoma. In this paper, we describe 2 pediatric cases where previously diagnosed dermoid cyst and dacrocystocele were found to be capillary hemangiomas upon biopsy. Approaches to distinguish capillary hemangiomas from other periocular tumors are further discussed. To our knowledge, this is the first case report of periocular hemangiomas imitating a dermoid cyst and a dacrocystocele. These cases emphasize the importance of including infantile hemangiomas in the differential diagnosis of subcutaneous periocular abnormalities.

  19. Solitary neurofibroma of eyelid masquerading as chalazion

    Directory of Open Access Journals (Sweden)

    Chen N

    2017-05-01

    Full Text Available Nancy Chen,1 Yung-Hsiang Hsu,2 Yuan-Chieh Lee1,3 1Department of Ophthalmology, Buddhist Tzu Chi General Hospital, Hualien, Taiwan; 2Department of Pathology, Buddhist Tzu Chi General Hospital, Hualien, Taiwan; 3Department of Ophthalmology and Visual Science, Tzu Chi University, Hualien, Taiwan Abstract: Neurofibroma, a benign peripheral nerve sheath tumor, usually appears together with café-au-lait spots, iris nodules, and other tumors within the scope of neurofibromatosis von Recklinghausen type 1 tumors. A solitary neurofibroma of the eyelid is relatively rare. In this case report, we present a 39-year-old woman who had a lesion on the eyelid crease, previously treated as a chalazion. Due to persistent wound crusting, the lesion was excised above the tarsus. Pathological examination revealed a solitary neurofibroma. The patient did not have other clinical symptoms of neurofibromatosis, and there was no recurrence of the nodule during the 1-year follow-up. Keywords: neurofibroma, eyelid tumor, chalazion

  20. Merkel cell carcinoma masquerading as a chalazion.

    Science.gov (United States)

    Rawlings, Nigel G; Brownstein, Seymour; Jordan, David R

    2007-06-01

    A 62-year-old woman presented with a large rapidly growing violaceous mass initially diagnosed as a chalazion. Histopathologic examination disclosed Merkel cell carcinoma (MCC). Radiotherapy was not tolerated. Despite chemotherapy, she succumbed to widespread metastases 13 months later. MCC must be included in the differential diagnosis of solitary eyelid nodules, requiring early and aggressive treatment.

  1. Solitary neurofibroma of eyelid masquerading as chalazion.

    Science.gov (United States)

    Chen, Nancy; Hsu, Yung-Hsiang; Lee, Yuan-Chieh

    2017-01-01

    Neurofibroma, a benign peripheral nerve sheath tumor, usually appears together with café-au-lait spots, iris nodules, and other tumors within the scope of neurofibromatosis von Recklinghausen type 1 tumors. A solitary neurofibroma of the eyelid is relatively rare. In this case report, we present a 39-year-old woman who had a lesion on the eyelid crease, previously treated as a chalazion. Due to persistent wound crusting, the lesion was excised above the tarsus. Pathological examination revealed a solitary neurofibroma. The patient did not have other clinical symptoms of neurofibromatosis, and there was no recurrence of the nodule during the 1-year follow-up.

  2. Periapical Infection Masquerading as a Nasal Pustule

    African Journals Online (AJOL)

    Ejide Dental. Complex, University of Benin. Teaching Hospital, P.M.B. 1111. Ugbowo, Benin City, Edo State,. Nigeria. E‑mail: clementazodo@yahoo.com. Introduction ... manifestation of periapical infection without an accompanying dental symptom may lead to ... glass ionomer cement and the patient was given a 1 month.

  3.  Masquerade of a Silent Killer

    Directory of Open Access Journals (Sweden)

    Padmini Venkataramani

    2011-09-01

    Full Text Available   Congenital long QT syndrome (LQTS, referred to as a ticking time-bomb is a cause of sudden death in young infants, children and adults.1 Its prevalence is estimated to be 1 in 2500 to 1 in 10,000 individuals internationally, with no racial predilection.2 It should be viewed as an unrecognized rather than a rare condition.1 This is a descriptive report of eight children diagnosed to have congenital LQTS from 2000 to 2007 (Table 1, in Sarawak General Hospital, Kuching, Sarawak, Malaysia, the main tertiary referral hospital for Sarawak. The population of Sarawak (2006 Census was 2,357,500 and that of Kuching, 435,000.

  4. Ameloblastic fibro-odontoma masquerading as odontoma

    Directory of Open Access Journals (Sweden)

    Kanwar Deep Singh Nanda

    2011-01-01

    Full Text Available Ameloblastic fibro-odontoma (AFO is a rare, benign epithelial mixed odontogenic tumor. It occurs as an intraosseous lesion, generally asymptomatic and more prevalent in children and adolescent. AFO is found on radiographic evaluation of patients with unerupted or impacted teeth in many cases. Histological examination reveals a fibrous soft tissue, islands of odontogenic epithelium and a disordered mixture of dental tissues. The treatment modality in most cases involves conservative surgery with enucleation. We present a case of 13-year-old boy with a missing right central incisor, mimicking-like odontoma on radiograph but proved to be AFO and treated with enucleation with preservation of impacted tooth. There was no recurrence after one year of follow-up. This report discusses the clinical, radiographical, histological features and surgical assessment to preserve the impacted tooth associated with AFO.

  5. Ameloblastic fibro-odontoma masquerading as odontoma.

    Science.gov (United States)

    Nanda, Kanwar Deep Singh; Marwaha, Mohita

    2011-01-01

    Ameloblastic fibro-odontoma (AFO) is a rare, benign epithelial mixed odontogenic tumor. It occurs as an intraosseous lesion, generally asymptomatic and more prevalent in children and adolescent. AFO is found on radiographic evaluation of patients with unerupted or impacted teeth in many cases. Histological examination reveals a fibrous soft tissue, islands of odontogenic epithelium and a disordered mixture of dental tissues. The treatment modality in most cases involves conservative surgery with enucleation. We present a case of 13-year-old boy with a missing right central incisor, mimicking-like odontoma on radiograph but proved to be AFO and treated with enucleation with preservation of impacted tooth. There was no recurrence after one year of follow-up. This report discusses the clinical, radiographical, histological features and surgical assessment to preserve the impacted tooth associated with AFO.

  6. Pheochromocytoma Masquerading as “Diabetic Ketoacidosis”

    Directory of Open Access Journals (Sweden)

    Jeffrey Stephen Hedley MD

    2016-04-01

    Full Text Available Diabetic ketoacidosis is a routinely encountered diagnosis in medicine. Physicians are trained early on to look for precipitants. Most clinicians assess for medication compliance, infection, ischemia, and the like. We present a case of pheochromocytoma presenting as “diabetic ketoacidosis.” The case serves as an example for broadening the differential diagnosis for patients with similar presentations. Additionally, the case helps inform our understanding of the so-called “stress reactions” that are commonly invoked in clinical rationale.

  7. Tubercular biliary stricture – a malignant masquerade

    African Journals Online (AJOL)

    2013-11-04

    Nov 4, 2013 ... chest radiograph and the results of blood tests were essentially normal ... basis of the imaging findings. Diagnostic ... stricture formation.[4] In this situation, comprehensive investigations usually fail to provide an accurate preoperative diagnosis. If the lesion appears to be resectable on imaging, surgery ...

  8. Severe hypothyroidism masquerading as renal impairment

    DEFF Research Database (Denmark)

    Bistrup, C; Jensen, Dorte Møller; Kvetny, J

    1996-01-01

    creatinine clearance and proteinuria, polymyositis with secondary glomerulopathy was suspected. Meanwhile, biopsies from skin, muscle, and kidney were normal. All symptoms disappeared 3 months after thyroid replacement therapy was initiated. S-TSH should be considered when evaluating patients with renal...

  9. Rare primary retroperitoneal teratoma masquerading as adrenal ...

    African Journals Online (AJOL)

    J.M. Ratkal

    Abstract. Objectives: To present a rare case of Primary mature cystic teratoma of right adrenal gland in adult female with an aim to review the published literature. Materials and Methods: The case details of a lady presenting with vague upper abdominal pain and on investigation was found to have a right adrenal mass were ...

  10. Giant solitary primary intracranial lymphoma masquerading as ...

    African Journals Online (AJOL)

    abp

    2017-11-01

    Nov 1, 2017 ... G-PCNSL-SG1 trial. Journal of Cancer Research and Clinical. Oncology. 2017; 143(9): 1815-21. PubMed | Google Scholar. 21. DeFilipp Z, Li S, El-Jawahri A, Armand P, Nayak L, Wang N et al. High-dose chemotherapy with thiotepa, busulfan and cyclophosphamide and autologous stem cell ...

  11. Orbital melanoma masquerading as a "Galloping haemangioma".

    Science.gov (United States)

    Lorenzano, Daniele; Miszkiel, Katherine; Rose, Geoffrey E

    2017-04-01

    We describe a 32-year-old pregnant woman who was referred to our clinic after 6 weeks of observation elsewhere with a rapidly expanding orbital mass, proptosed globe and slowly decreasing of vision in her left eye. To our examination the patient presented with congested optic disc fine macular striae and some slight choroidal elevation without any retinal pigmentation. An MRI scan without contrast was performed, suggesting the signal charactheristics of an orbital mass consistent with a cellular lesion such as a cavernous hemangioma or a solitary fibrous tumour. Despite the benign-looking imaging, the fast-growing pattern of the lesion suggested a more sinister picture. A fine needle aspiration of the orbital mass was carried out, revealing the presence of an amelanotic melanoma. This case highlights the importance of considering the presence of a melanoma when there is a clear history of a rapid lesion progression.

  12. Double sigmoid atresia with meconium pseudocyst masquerading ...

    African Journals Online (AJOL)

    stump was brought out as a stoma and the atretic bowel left in situ. The child was discharged in satisfactory ... patient care, drafting the manuscript; Monika Bawa: supervision, patient in charge, finalizing the manuscript;. Raj Kumar: patient care and intraoperative assistance;. KLN Rao: supervision, finalizing the manuscript.

  13. The African Renaissance: Myth, Mask or Masquerade ...

    African Journals Online (AJOL)

    The African Renaissance, now a familiar theme in South Africa, has a longer history than general discourse seems to suggest. In the past, it has been approached from different perspectives: the political, ideological, and cultural. The African Renaissance was originally the ideology of Pan-Africanism. It was an ideological ...

  14. Ureteral valve masquerading as obstructive megaureter

    Directory of Open Access Journals (Sweden)

    Sushmita Bhatnagar

    2017-05-01

    Full Text Available Primary obstructed megaureter is a common urological condition in the pediatric age group. It is one of the differentials for congenital anomalies of the kidney and urinary tract that include a myriad of structural anomalies of the urinary tract. The obstructive conditions are usually managed conservatively unless there is deterioration in renal function, repeated urinary tract infection, or any other symptom such as abdominal pain on ipsilateral side and hypertension. Presented here is a case of left lower ureteral valve that was diagnosed as primary obstructed megaureter with pain in abdomen and reduced ipsilateral renal function wherein ureteral valve was detected incidentally intraoperatively. Excision of the valve with end to end uretero-ureteral anastomoses over a D-J stent, without resorting to ureteric reimplantation was successful in relieving the obstruction and hydronephrosis

  15. Lipedema--the non-lymphatic masquerader.

    Science.gov (United States)

    Beninson, J; Edelglass, J W

    1984-08-01

    Two cases of lipedema are presented. They illustrate this clinical syndrome which occurs almost exclusively in women and presents as grossly enlarged legs, thighs and buttocks. The etiology remains uncertain. Although infrequently diagnosed, lipedema is not rare. We report success treating such patients with properly measured and fitted compression garments.

  16. Pseudomonas aeruginosa endophthalmitis masquerading as chronic uveitis

    Directory of Open Access Journals (Sweden)

    Kalpana Badami Nagaraj

    2013-01-01

    Full Text Available A 65-year-old male presented with decreased vision in the left eye of 15-day duration after having undergone an uneventful cataract surgery 10 months back. He had been previously treated with systemic steroids for recurrent uveitis postoperatively on three occasions in the same eye. B-scan ultrasonography showed multiple clumplike echoes suggestive of vitreous inflammation. Aqueous tap revealed Pseudomonas aeruginosa sensitive to ciprofloxacin. The patient was treated with intravitreal ciprofloxacin and vancomycin along with systemic ciprofloxacin with good clinical response. Even a virulent organism such as P.aeruginosa can present as a chronic uveitis, which, if missed, can lead to a delay in accurate diagnosis and appropriate management.

  17. Rare primary retroperitoneal teratoma masquerading as adrenal ...

    African Journals Online (AJOL)

    Objectives: To present a rare case of Primary mature cystic teratoma of right adrenal gland in adult female with an aim to review the published literature. Materials and Methods: The case details of a lady presenting with vague upper abdominal pain and on investigation was found to have a right adrenal mass were collected ...

  18. Fixed Cutaneous Sporotrichosis Masquerading As Lupus Vulgaris

    Directory of Open Access Journals (Sweden)

    Ravikumar B C

    1998-01-01

    Full Text Available Sporotrichosis is reported to be rare in India. Fixed cutaneous variety is the most pleomorphic type of sporotrichosis and it may mimick several other dermatoses resulting in difficulty in making a correct diagnosis. Here we are reporting a case of fixed cutaneous sporotrichosis which resembled lupus vulgaris both clinically and histopathologically.

  19. Ameloblastic fibro-odontoma masquerading as odontoma

    OpenAIRE

    Kanwar Deep Singh Nanda; Mohita Marwaha

    2011-01-01

    Ameloblastic fibro-odontoma (AFO) is a rare, benign epithelial mixed odontogenic tumor. It occurs as an intraosseous lesion, generally asymptomatic and more prevalent in children and adolescent. AFO is found on radiographic evaluation of patients with unerupted or impacted teeth in many cases. Histological examination reveals a fibrous soft tissue, islands of odontogenic epithelium and a disordered mixture of dental tissues. The treatment modality in most cases involves conservative surgery w...

  20. Severe hypothyroidism masquerading as renal impairment

    DEFF Research Database (Denmark)

    Bistrup, C; Jensen, Dorte Møller; Kvetny, J

    1996-01-01

    A case of severe hypothyroidism in a 51-year old male is presented. The patient was especially complaining of weakness, stiffness and moderate pain in the proximal muscle groups together with rhinorrhea and nasal stenosis. Because of severely elevated S-creatine-kinase combined with reduced...